MedWorm.com provides a medical RSS filtering service. Over 6000 RSS medical sources are combined and output via different filters. This feed contains the latest items from the 'Blood Reviews' source. Wed, 08 Feb 2012 17:41:58 +0100 http://www.medworm.com/index.php?rid=5660581&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000828%2Fabstract%3Frss%3Dyes Abstract: Blood transfusion is indispensable for modern medicine. In developed countries, the blood supply is adequate and safe but blood for alloimmunized patients is often unavailable. Concerns are increasing that donations may become inadequate in the future as the population ages prompting a search for alternative transfusion products. Improvements in culture conditions and proof-of-principle studies in animal models have suggested that ex-vivo expanded red cells may represent such a product. Compared to other cell therapies transfusion poses the unique challenge of requiring great cell doses (2.5×1012 cells vs 107 cells). Although production of such cell numbers is theoretically possible, current technologies generate red cells in numbers sufficient only for safety studies. It is con... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5660581 Mon, 19 Dec 2011 05:00:00 +0100 5660581 http://www.medworm.com/index.php?rid=5660580&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000816%2Fabstract%3Frss%3Dyes Abstract: Advances in therapy can essentially be measured using two parameters; introduction of a new agent which benefits an increased number of patients over prevailing treatments or more selective use of an existing drug by matching it to the biologic characteristics associated with response. In reviewing the therapeutic landscape of myelodysplastic syndromes (MDS), both should be applied to gauge the advances in therapy. While several new drugs are currently in clinical trials for the treatment of MDS, three drugs were approved for use in the last decade and sufficient time has elapsed to take stock of the benefit they have produced in the outcome of patients both in terms of survival and quality of life. For the two hypomethylating agents, response remains limited to 50% patients at b... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5660580 Fri, 02 Dec 2011 05:00:00 +0100 5660580 http://www.medworm.com/index.php?rid=5660579&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000804%2Fabstract%3Frss%3Dyes Abstract: Secondary anaemia or the anaemia of chronic disease (ACD) is the commonest form of anaemia in hospitalised patients and the second most prevalent anaemia worldwide after iron deficiency. It is characterised by defective iron incorporation in erythropoiesis, an impaired response to erythropoietin, a decrease in erythropoietin production and cytokine induced shortening of red cell survival.For many patients with ACD the cause is apparent but for many others the underlying disease needs to be determined and such patients are often referred to haematologists for investigation. The search for the cause can be a fascinating exercise in good history taking, examination skills and performing and interpreting appropriate investigations. This review covers the pathogenesis and causes of AC... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5660579 Fri, 02 Dec 2011 05:00:00 +0100 5660579 http://www.medworm.com/index.php?rid=5660578&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000701%2Fabstract%3Frss%3Dyes Abstract: For many years, programmed cell death, known as apoptosis, was attributed exclusively to nucleated cells. Currently, however, apoptosis is also well-documented in anucleate platelets. This review describes extrinsic and intrinsic pathways of apoptosis in nucleated cells and in platelets, platelet apoptosis induced by multiple chemical stimuli and shear stresses, markers of platelet apoptosis, mitochodrial control of platelet apoptosis, and apoptosis mediated by platelet surface receptors PAR-1, GPIIbIIIa and GPIbα. In addition, this review presents data on platelet apoptosis provoked by aging of platelets in vitro during platelet storage, platelet apoptosis in pathological settings in humans and animal models, and inhibition of platelet apoptosis by cyclosporin A, intravenous im... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5660578 Mon, 07 Nov 2011 05:00:00 +0100 5660578 http://www.medworm.com/index.php?rid=5362706&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000786%2Fabstract%3Frss%3Dyes (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5362706 Tue, 01 Nov 2011 04:00:00 +0100 5362706 http://www.medworm.com/index.php?rid=5495505&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000695%2Fabstract%3Frss%3Dyes Abstract: Generation of inhibitory antibodies upon repeated FVIII infusion represents a major complication in hemophilia care. Professional antigen presenting cells (APCs) are crucial for orchestration of humoral immune responses. APCs are capable of internalizing soluble as well as particulate antigens through various mechanisms resulting in loading of antigen-derived peptides on MHC class I or II for presentation to T cells. This review highlights how FVIII is recognized and processed by APCs. The significance and contribution of candidate receptors involved in FVIII uptake by APC are discussed. Recent findings defining the repertoire of FVIII peptides presented on MHC class II are addressed. Studies in murine models of hemophilia A suggest that modulation of APC function can reduce inhi... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5495505 Mon, 31 Oct 2011 04:00:00 +0100 5495505 http://www.medworm.com/index.php?rid=5495504&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000683%2Fabstract%3Frss%3Dyes We present a comprehensive review on adverse events addressing all types of blood donation including whole blood, plasma, platelet, peripheral blood stem cell, leucocyte and bone marrow donation. In addition, we outline strategies for the prevention and treatment of these events and give a blueprint for future research in this field. (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5495504 Thu, 13 Oct 2011 04:00:00 +0100 5495504 http://www.medworm.com/index.php?rid=5362705&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X1100066X%2Fabstract%3Frss%3Dyes Abstract: Chronic myeloid leukemia (CML) is a myeloproliferative disorder that affects 5000 new patients per year in the United States. Prior to 10years ago, durable remission was rare and patients often underwent bone marrow transplantation with substantial morbidity and mortality. Fortunately, CML has been the epicenter of exciting advances in cancer therapy with the discovery of the Bcr–Abl gene fusion and the subsequent development of imatinib mesylate, a small molecule tyrosine kinase inhibitor, to target the kinase activity of the bcr–abl protein product. Despite unprecedented durability for complete hematologic, cytogenetic, and molecular responses seen with front-line imatinib therapy, many patients require alternative therapy because of drug intolerance, suboptimal response, p... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5362705 Mon, 10 Oct 2011 04:00:00 +0100 5362705 http://www.medworm.com/index.php?rid=5495503&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000658%2Fabstract%3Frss%3Dyes Abstract: ALL blast cells express a variety of specific antigens e.g. CD19, CD20, CD22, CD33, and CD52, which serve as targets for Monoclonal Antibodies (MoAbs). So far, the most experience is available for anti-CD20 (rituximab), which has been combined with chemotherapy for treatment of mature B-ALL/Burkitt's lymphoma. Studies with rituximab have also been completed in B-precursor ALL. Another antigen, CD19, is of great interest due to a very high rate of expression in ALL. It can be targeted by a bispecific monoclonal antibody, Blinatumomab, directed against CD19 and CD3. Smaller studies or case reports are also available for the anti CD52 antibody (Alemtuzumab), for anti CD22 (Epratuzumab) or anti CD33 (Gemtuzumab). Available data demonstrate that MoAb therapy in ALL is a highly promisi... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5495503 Fri, 30 Sep 2011 04:00:00 +0100 5495503 http://www.medworm.com/index.php?rid=5495502&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000671%2Fabstract%3Frss%3Dyes Abstract: Chronic lymphocytic leukemia (CLL) is a disease of older patients and median age at diagnosis is 72years. This older group is under-represented in clinical trials, (median age 58–62years). Here we review background data on incidence, survival, definitions of older age, fitness criteria, frailty and co-morbidities. Issues influencing the choice of therapy in older patients are also addressed and different therapeutic options are highlighted based on recent available data. Fit older patients with less co-morbidities benefit most from the very effective chemoimmunotherapy (FC-R) given for younger patients today, but whether other novel drug combinations or new agents are more suitable for less fit patients is still unsettled. Based on careful evaluation of published data from larg... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5495502 Wed, 28 Sep 2011 04:00:00 +0100 5495502 http://www.medworm.com/index.php?rid=5495501&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000543%2Fabstract%3Frss%3Dyes Abstract: Vitamin K antagonists (VKA) have been the mainstay of oral anticoagulant therapy for over 60years. In this review we critically assess the evidence for the importance of vitamin K nutrition during VKA therapy; the methodologies for measuring dietary intakes; the vitamin K intake data in patients on VKA and healthy people; and the experimental evidence for the influence of vitamin K intakes and biochemical measures of vitamin K status on VKA response. Several studies show that dietary intakes of phylloquinone (vitamin K1) are associated to the sensitivity and stability of anticoagulation during initiation and maintenance dosing with low habitual intakes associated with greater instability of the INR and risk of sub-therapeutic anticoagulation. Preliminary evidence suggests that th... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5495501 Wed, 14 Sep 2011 04:00:00 +0100 5495501 http://www.medworm.com/index.php?rid=5362704&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000531%2Fabstract%3Frss%3Dyes This article reviews these contrasting problems and discusses strategies for prevention and management in the context of renal impairment, renal replacement therapy and renal transplantation. (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5362704 Mon, 29 Aug 2011 04:00:00 +0100 5362704 http://www.medworm.com/index.php?rid=5362703&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X1100052X%2Fabstract%3Frss%3Dyes Abstract: Telomeres are long (TTAGGG)n nucleotide repeats and an associated protein complex located at the end of the chromosomes. They shorten with every cell division and, thus are markers for cellular aging, senescence, and replicative capacity. Telomere dysfunction is linked to several bone marrow disorders, including dyskeratosis congenita, aplastic anemia, myelodysplastic syndrome, and hematopoietic malignancies. Hematopoietic stem cell transplantation (HSCT) provides an opportunity in which to study telomere dynamics in a high cell proliferative environment. Rapid telomere shortening of donor cells occurs in the recipient shortly after HSCT; the degree of telomere attrition does not appear to differ by graft source. As expected, telomeres are longer in recipients of grafts with long... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5362703 Mon, 18 Jul 2011 04:00:00 +0100 5362703 http://www.medworm.com/index.php?rid=5362702&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000518%2Fabstract%3Frss%3Dyes Abstract: Since the first human cord blood transplant, performed in 1988, cord blood banks have been established worldwide for collection and cryopreservation of cord blood for allogeneic hematopoietic stem cell transplant. Umbilical cord blood (UCB) has now become one of the most commonly used source of hematopoietic stem cells for allogeneic transplantation. Today a global network of cord blood banks and transplant centers has been established for a common inventory with an estimated 600,000 UCB have been banked and more than 20,000 UCB units distributed worldwide for adults and children with severe hematological diseases. Several studies have shown that the number of cells is the most important factor for engraftment while some degree of HLA mismatches is acceptable. The absence of ethi... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5362702 Mon, 18 Jul 2011 04:00:00 +0100 5362702 http://www.medworm.com/index.php?rid=5145041&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000506%2Fabstract%3Frss%3Dyes Abstract: JAK-STAT is an appealing but also problematic drug target in BCR-ABL1-negative myeloproliferative neoplasms (MPN) – it is appealing because the majority of patients with MPN harbor gain-of-function JAK2 or MPL mutations – it is problematic because currently available JAK inhibitors do not distinguish between oncogenic and physiologic JAK-STAT activation. Furthermore, JAK-STAT-relevant mutations in MPN do not always constitute the predominant or ancestral mutant clone. Such complexities undermine the value of JAK-STAT as a robust drug target in MPN and partly explain the hitherto lack of histologic or molecular remissions associated with currently available JAK inhibitors. Most of these drugs were, however, effective in alleviating constitutional symptoms and reducing spleen s... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5145041 Sun, 10 Jul 2011 23:00:00 +0100 5145041 http://www.medworm.com/index.php?rid=5362701&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X1100049X%2Fabstract%3Frss%3Dyes Abstract: Acute myeloid leukemia (AML) carrying nucleophosmin (NPM1) mutations displays distinct molecular and clinical–pathological features that led to its inclusion as provisional entity in 2008 WHO classification of myeloid neoplasms. Since NPM1 mutations behave as a founder genetic lesion in AML, they could be an attractive target for therapeutic intervention. Here, we discuss the potential for developing targeted therapies for NPM1-mutated AML with focus on: i) interfering with the abnormal traffic of the NPM1 leukemic mutant, i.e. its cytoplasmic dislocation; ii) disrupting the nucleolar structure/function by interfering with residual wild-type nucleophosmin and other nucleolar components acting as hub proteins; and iii) evaluating the activity of epigenetic drugs (e.g. 5-azacytid... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5362701 Mon, 04 Jul 2011 04:00:00 +0100 5362701 http://www.medworm.com/index.php?rid=5362700&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000361%2Fabstract%3Frss%3Dyes Abstract: Several prospective epidemiological studies and clinical observations provided evidence regarding fibrinogen and coronary artery disease (CAD). Many of these studies firmly correlate fibrinogen with CAD. However, it is uncertain whether this relation is causal or reflects genetic variability and residual confounding by other risk factors. Several polymorphisms on fibrinogen chain genes affect its levels, however only few of the genetic variants are associated with increased cardiovascular risk. As regards the role of fibrinogen in myocardial infarction (MI) studies indicate that genetic variations have at best a modest impact on the process resulting in MI. Therefore, the screening of fibrinogen genes might not be useful for the assessment of the risk of MI. However, the findings... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5362700 Wed, 08 Jun 2011 04:00:00 +0100 5362700 http://www.medworm.com/index.php?rid=5145040&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000373%2Fabstract%3Frss%3Dyes Abstract: Allogeneic transplantation is established as a curative treatment for follicular lymphoma, but with considerable short and long-term morbidity and mortality. Data and controversies regarding conditioning regimen, donor source, GVHD prophylaxis, post transplant interventions and approaches to predict and reduce morbidity and mortality are reviewed. Total body irradiation is very effective but toxic and reduced intensity conditioning is often preferred though associated with somewhat higher rates of recurrence. The risk of chronic GVHD and its late sequelae can be markedly reduced by in-vivo T-cell depletion using alemtuzumab but also leads to somewhat higher incidence of disease recurrence. When using such treatment strategies, one can consider prophylactic or preemptive donor lym... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5145040 Sun, 05 Jun 2011 23:00:00 +0100 5145040 http://www.medworm.com/index.php?rid=5145037&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X1100021X%2Fabstract%3Frss%3Dyes Abstract: Mutational reports over the past two decades have accumulated an immense amount of literature for inherited Factor XIII deficiency. However, the genotype and phenotype correlations for inherited Factor XIII deficiency are complicated. While many studies clearly prove a cause and effect relationship for the reported mutations, others are lacking in this regard. The F13B gene remains an elusive component as far as inherited Factor XIII deficiencies are concerned. Also, an in-depth analysis into the heterozygous state of this deficiency is also lacking. In this review we have tried to analyze and present an exhaustive amount of mutational data from the past three decades. The source of our mutational data is our website dedicated to Factor XIII deficiencies (www.F13-database.de) as ... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5145037 Sun, 05 Jun 2011 23:00:00 +0100 5145037 http://www.medworm.com/index.php?rid=5145039&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X1100035X%2Fabstract%3Frss%3Dyes Abstract: The incidence of venous thromboembolism (VTE), including deep vein thrombosis and pulmonary embolism, is increasing and the disease has been found to account for over 500,000 annual deaths in the European Union. VTE is associated with increased mortality and may lead to serious long-term complications. Unfractionated heparin (UFH), low molecular weight heparin (LMWH) and vitamin K antagonists (VKA) have remained standard of care for many years. Recent trials of novel anticoagulants have indicated that new therapeutical options may soon become available. Studies on the role of new agents in VTE prevention in patients undergoing orthopaedic surgery have provided the evidence suggesting potential value of these drugs for the management of acute events. At present, investigation of n... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5145039 Tue, 24 May 2011 23:00:00 +0100 5145039 http://www.medworm.com/index.php?rid=5145038&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000336%2Fabstract%3Frss%3Dyes Abstract: Diagnosis of haemoglobinopathies, including thalassaemias, can result from either a clinical suspicion of a disorder of globin chain synthesis or from follow-up of an abnormality detected during screening. Screening may be carried out as part of a well defined screening programme or be an ad hoc or opportunistic test. Screening may be preoperative, neonatal, antenatal, preconceptual, premarriage or targeted at specific groups perceived to be at risk. Screening in the setting of haemoglobinopathies may be directed at optimising management of a disorder by early diagnosis, permitting informed reproductive choice or preventing a serious disorder by offering termination of pregnancy. Diagnostic methods and algorithms will differ according to the setting. As the primary test, high per... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=5145038 Thu, 19 May 2011 23:00:00 +0100 5145038 http://www.medworm.com/index.php?rid=4904922&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000348%2Fabstract%3Frss%3Dyes Abstract: Schistosomes (blood flukes) are long lived, intravascular parasites that afflict ~200 million people worldwide. Here we review the potential ability of these parasites to exert control on local vascular physiology. We examine schistosome kallikrein-like proteins that drive vasodilation. We review biogenic amine metabolism in the parasites that involve the vasodilator histamine and its receptors and the vasoconstrictor serotonin and its receptor. Schistosomes can trigger the release of histamine from host cells and can import serotonin. We consider the ability of schistosomes to generate and release the eicosanoid vasodilators PGD2 and PGE2 and the vasoconstrictors LTB4 and LTC4. The literature on nitric oxide metabolism in these blood flukes is assessed. Finally the potential imp... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4904922 Wed, 04 May 2011 23:00:00 +0100 4904922 http://www.medworm.com/index.php?rid=4904923&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000324%2Fabstract%3Frss%3Dyes This article will provide practical guidance on the management of bortezomib-, thalidomide-, and lenalidomide-associated AEs, to maximize treatment feasibility and active drug delivered, and thus help minimize toxicity and maximize outcomes. (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4904923 Sun, 17 Apr 2011 23:00:00 +0100 4904923 http://www.medworm.com/index.php?rid=4904921&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000312%2Fabstract%3Frss%3Dyes Abstract: Platelet function disorders are inherited and acquired conditions that represent a common cause of bleeding. Their clinical findings are generally similar to von Willebrand disease. It is often challenging to diagnose common platelet function disorders due to heterogeneity in their features, uncertainties about their pathogenesis and genetic cause, variability in the procedures used to assess platelet function in diagnostic laboratories and the lack of diagnostic criterion. Some inherited platelet function disorders have been established to increase risks for bleeding and bleeding scores. However, bleeding history assessment tools are not validated for use in diagnosing platelet function disorders. Standardized tests that assess aggregation function, dense granule deficiency and ... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4904921 Sun, 17 Apr 2011 23:00:00 +0100 4904921 http://www.medworm.com/index.php?rid=4904919&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000233%2Fabstract%3Frss%3Dyes Abstract: Almost thirty years has passed since the description of platelet-type von Willebrand disease. Named initially pseudo-von Willebrand disease, this mild mucocutaneous bleeding disorder has an intrinsic defect in platelets rather than VWF. Due to its unique features and its similarity to the more common type 2B VWD, many investigators were intrigued by its nature, pathophysiology and molecular genetics aspects. The challenges to diagnose this disorder have created debate in literature about the best method of discrimination from type 2B VWD. The recent development of the PT-VWD mouse model carries huge potential for further understanding of the disease as well as the platelet defect and its influence on other biological processes. This review provides a basic as well as updated know... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4904919 Sun, 17 Apr 2011 23:00:00 +0100 4904919 http://www.medworm.com/index.php?rid=4904920&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000221%2Fabstract%3Frss%3Dyes Abstract: When platelet numbers are low or when their function is disabled, the risk of bleeding is high, which on the one hand indicates that in normal life vascular damage is a rather common event and that hence the role of platelets in maintaining a normal hemostasis is a continuously ongoing physiological process. Upon vascular injury, platelets instantly adhere to the exposed extracellular matrix resulting in platelet activation and aggregation to form a hemostatic plug. This self-amplifying mechanism nevertheless requires a tight control to prevent uncontrolled platelet aggregate formation that eventually would occlude the vessel. Therefore endothelial cells produce inhibitory compounds such as prostacyclin and nitric oxide that limit the growth of the platelet thrombus to the damage... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4904920 Thu, 14 Apr 2011 23:00:00 +0100 4904920 http://www.medworm.com/index.php?rid=4672278&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000208%2Fabstract%3Frss%3Dyes Abstract: Recurrent losses or gains of genomic material as well as mutations of key tumor suppressors (ATM and TP53) have been identified in chronic lymphocytic leukemia (CLL). These aberrations are important “drivers” of the disease and some of its clinical characteristics. There is a remarkable heterogeneity in the clinical course between patient subgroups with distinct genetic features. While some mutations are associated with poor outcome (particularly 17p− and TP53 mutation and to a lesser extend 11q−) others are linked to a favorable outcome (13q− as sole aberration; mutated IGHV). Our improved understanding of the clinical course of specific genetic subgroups is beginning to be translated into genotype specific treatment approaches where genetic subgroups (e.g. 17p−) are... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4672278 Thu, 24 Mar 2011 00:00:00 +0100 4672278 http://www.medworm.com/index.php?rid=4672279&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000191%2Fabstract%3Frss%3Dyes Abstract: The International Randomized Study of Interferon vs. STI571 (IRIS) trial that investigated the use of the tyrosine kinase inhibitor (TKI) imatinib (versus interferon) changed the treatment and outcome of chronic myeloid leukemia (CML). Long-term follow-up of IRIS patients has defined response parameters and methods of tracking residual disease with cytogenetic testing of bone marrow metaphases and molecular monitoring of BCR–ABL transcripts using quantitative reverse-transcriptase polymerase chain reaction. Cytogenetic and molecular responses are now considered useful surrogates for long-term outcome. Early and robust response to imatinib predicts positive long-term outcomes. However, 15–25% of patients fail initial treatment or become intolerant of imatinib and need increase... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4672279 Fri, 18 Mar 2011 00:00:00 +0100 4672279 http://www.medworm.com/index.php?rid=4672277&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000087%2Fabstract%3Frss%3Dyes Abstract: Antiplatelet and anticoagulant drugs are effective for the prevention of arterial and venous thrombosis but patients continue to experience major cardiovascular events despite their use. Strategies to improve the effectiveness of antithrombotic therapies include selecting the optimal drug and dosing regimen, the use of combinations of antiplatelet and anticoagulant drugs and the development of new more effective drugs to replace existing therapies. Evidence from randomized controlled trials indicates that the combination of aspirin and an anticoagulant is more effective than aspirin alone for the prevention of recurrent cardiovascular events in patients with acute coronary syndrome and is more effective than anticoagulation alone for the prevention of thromboembolic events in pat... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4672277 Mon, 28 Feb 2011 00:00:00 +0100 4672277 http://www.medworm.com/index.php?rid=4672276&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000075%2Fabstract%3Frss%3Dyes Abstract: Platelet transfusions are mainly used for patients with thrombocytopenia due to bone marrow failure, especially cancer patients developing severe chemotherapy-induced thrombocytopenia (e.g. patients with acute leukemia or other hematologic malignancies). A prophylactic transfusion strategy is now generally accepted in developed countries. Some clinical data, however, support the use of a therapeutic transfusion strategy at least for certain subsets of these patients. Several methodological approaches can then be used to evaluate the outcome of platelet transfusions, including peripheral blood platelet increments and bleeding assessments. Several factors will influence the efficiency of platelet transfusions; fever and ongoing hemorrhage are among the most important patient-depend... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4672276 Mon, 14 Feb 2011 00:00:00 +0100 4672276 http://www.medworm.com/index.php?rid=4596502&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000597%2Fabstract%3Frss%3Dyes Abstract: For many years, the oral combination melphalan–prednisone (MP) has been considered the standard of care for patients with multiple myeloma (MM) not eligible for autologous stem cell transplantation. In the era of novel agents, the introduction of immunomodulatory drugs and proteasome inhibitors has challenged the role of MP and led to new standards of care for this disease. Five randomized phase III studies compared the traditional MP with the MP plus thalidomide (MPT). All these studies showed a prolonged time to progression (TTP) with the 3-drug combination. However, in only two of these trials this advantage translated into an improvement in overall survival (OS). In another randomized trial, MP plus bortezomib (VMP) was correlated with an increase in both TTP and OS compare... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4596502 Tue, 08 Feb 2011 00:00:00 +0100 4596502 http://www.medworm.com/index.php?rid=4672275&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000063%2Fabstract%3Frss%3Dyes Abstract: Plasma cell leukemia (PCL) is a rare, yet aggressive plasma cell (PC) neoplasm, variant of multiple myeloma (MM), characterized by high levels of PCs circulating in the peripheral blood. PCL can either originate de novo (primary PCL) or as a secondary leukemic transformation of MM (secondary PCL). Presenting signs and symptoms are similar to those seen in MM such as renal insufficiency, hypercalcemia, lytic bone lesions, anemia, and thrombocytopenia, but can also include hepatomegaly and splenomegaly. The diagnostic evaluation of a patient with suspected PCL should include a review of the peripheral blood smear, bone marrow aspiration and biopsy, serum protein electrophoresis (SPEP) with immunofixation, and protein electrophoresis of an aliquot from a 24h urine collection (UPEP).... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4672275 Fri, 04 Feb 2011 00:00:00 +0100 4672275 http://www.medworm.com/index.php?rid=4596505&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X11000026%2Fabstract%3Frss%3Dyes In conclusion, quantitative MRD monitoring seems to be mandatory to assure safe and effective immunotherapy in the context of alloSCT for CLL, which should, however, be best performed within clinical studies. (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4596505 Thu, 27 Jan 2011 00:00:00 +0100 4596505 http://www.medworm.com/index.php?rid=4596506&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000780%2Fabstract%3Frss%3Dyes Abstract: The diagnosis of antiphospholipid syndrome is predominantly made in the laboratory and depends on the persistent presence of antiphospholipid antibodies in individuals with thrombosis or pregnancy morbidity. Correct diagnosis of the syndrome is imperative to prevent unnecessary long secondary thromboprophylaxis. Three antiphospholipid antibody subtypes are included in the classification criteria of the antiphospholipid syndrome: lupus anticoagulants, anticardiolipin antibodies and anti-β2-glycoprotein I antibodies. Only lupus anticoagulants are undisputedly associated with thrombosis, which is why the serological criteria of the antiphospholipid syndrome are under debate. All of the assays used to detect antiphospholipid antibodies are in need of better standardization, although... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4596506 Tue, 18 Jan 2011 00:00:00 +0100 4596506 http://www.medworm.com/index.php?rid=4596503&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000706%2Fabstract%3Frss%3Dyes Abstract: Myeloid leukemias are clonal disorders originating in a primitive multipotential hematopoietic cell and characterized by aberrant proliferation, differentiation and maturation of leukemic progenitors and precursor cells. These diseases are the result of multiple genetic and epigenetic events, although the nature and number of events vary widely among patients. For over four decades, studies have identified sub-populations of leukemic cells possessing different functional capabilities. Investigators have struggled to understand the origin and significance of this heterogeneity. The stem cell model for myeloid malignancies has offered one potential explanation. Since 1994, experimental data supporting the presence of leukemia stem cells has been reported and validated in numerous s... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4596503 Mon, 10 Jan 2011 00:00:00 +0100 4596503 http://www.medworm.com/index.php?rid=4596501&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000603%2Fabstract%3Frss%3Dyes Abstract: Physicians treating patients with the classic Philadelphia-negative myeloproliferative neoplasms (Ph-negative MPNs) (polycythemia vera [PV], essential thrombocythemia [ET] and primary myelofibrosis [PMF]) traditionally had few therapeutic drugs available. Spurred by the discovery of activating mutation of the JAK2 tyrosine kinase (JAK2 V617F mutation) in patients with Ph-negative MPNs several years ago, several JAK2 inhibitors were synthesized and are currently undergoing clinical trials in patients with PMF, PV and ET. Initial results from these studies have shown that these drugs can markedly reduce spleen size and alleviate constitutional symptoms, increase weight and improve exercise capacity in MF patients, thus improve quality of their life, which is significant clinical be... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4596501 Mon, 22 Nov 2010 00:00:00 +0100 4596501 http://www.medworm.com/index.php?rid=4254669&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000445%2Fabstract%3Frss%3Dyes Abstract: The management of chronic lymphocytic leukaemia is currently undergoing profound changes. Several drugs like bendamustine, alemtuzumab and rituximab have recently been approved for CLL treatment by regulatory agencies. New and very promising compounds like lenalidomide, ofatumumab, GA101, flavopiridol, or ABT-263 are currently investigated in clinical trials and are likely to further enlarge the therapeutic armamentarium in the next years. Latest results show that chemoimmunotherapies like FCR (fludarabine, cyclophosphamide and rituximab) may improve the life expectancy of CLL patients. This new paradigm will modify the way of CLL management in a radical manner. Finally, the development of new biological markers that describe distinct forms of CLL allows to enter the era of perso... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4254669 Mon, 22 Nov 2010 00:00:00 +0100 4254669 http://www.medworm.com/index.php?rid=4254673&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000494%2Fabstract%3Frss%3Dyes Abstract: Acute myeloid leukaemia (AML) is one of the most common haematological malignancies and is increasing in frequency due to an ageing population. Whilst remission will be achieved in up to 80% of those receiving intensive chemotherapy, the main variables precluding cure are the treatment-related mortality and relapse rates. Decisions on intensification, de-escalation and allografting rely on the ability to divide an apparently homogeneous group according to risk. A wide range of clinical, cytogenetic and molecular variables may be used to inform this task. Cytogenetic and molecular characterisation has already identified subgroups, such as acute promyelocytic leukaemia (APL) with t(15;17)/PML-RARA and AML with FLT3 mutation for which targeted therapies are available, and further mo... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4254673 Tue, 16 Nov 2010 00:00:00 +0100 4254673 http://www.medworm.com/index.php?rid=4160581&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000585%2Fabstract%3Frss%3Dyes (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4160581 Mon, 01 Nov 2010 00:00:00 +0100 4160581 http://www.medworm.com/index.php?rid=4160580&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000573%2Fabstract%3Frss%3Dyes (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4160580 Mon, 01 Nov 2010 00:00:00 +0100 4160580 http://www.medworm.com/index.php?rid=4254671&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000469%2Fabstract%3Frss%3Dyes Abstract: The myelodysplastic syndromes (MDS) are characterized by cytopenias and acute myeloid leukemia risk. Most MDS patients eventually require transfusion of red blood cells for anemia, placing them at risk of iron overload (IOL). In beta-thalassemia major, transfusional IOL leads to organ dysfunction and death, however, with iron chelation therapy survival improved to near normal and organ function was improved. In lower risk MDS, several non-randomized studies suggest an adverse effect of IOL on survival, and that lowering iron minimizes this impact and may improve organ function. While guidelines for MDS generally recommend chelation in selected lower risk patients, data are emerging suggesting IOL may impact adversely on the outcome of higher risk MDS and stem cell transplantation... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4254671 Fri, 29 Oct 2010 00:00:00 +0100 4254671 http://www.medworm.com/index.php?rid=4254670&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000482%2Fabstract%3Frss%3Dyes Abstract: Venous access is an essential aspect of hemophilia care. When peripheral venipuncture is not feasible, central venous access devices (CVADs) or the creation of an arteriovenous fistula (AVF) allow the infusion of clotting factor concentrate to treat or prevent bleeding events or eliminate inhibitors. Infection is the primary complication associated with CVADs and the most common reason for their removal. Complications of AVF include occlusion and limb length discrepancies.Research Highlights: ►Evaluate the appropriate use, selection, and placement of central venous access devices (CVADs) in patients with hemophilia. ►Examine the roles of education, maintenance, and monitoring in optimizing CVAD use. ►Review CVAD-related complications and strategies for their prevention. ►... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4254670 Thu, 28 Oct 2010 00:00:00 +0100 4254670 http://www.medworm.com/index.php?rid=4596504&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000470%2Fabstract%3Frss%3Dyes Abstract: T-cell acute lymphoblastic leukemia (T-ALL) is an aggressive hematologic tumor resulting from the malignant transformation of immature T-cell progenitors. Originally associated with a dismal prognosis, the outcome of T-ALL patients has improved remarkably over the last two decades as a result of the introduction of intensified chemotherapy protocols. However, these treatments are associated with significant acute and long-term toxicities, and the treatment of patients presenting with primary resistant disease or those relapsing after a transient response remains challenging.T-ALL is a genetically heterogeneous disease in which numerous chromosomal and genetic alterations cooperate to promote the aberrant proliferation and survival of leukemic lymphoblasts. However, the identifica... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4596504 Thu, 21 Oct 2010 00:00:00 +0100 4596504 http://www.medworm.com/index.php?rid=4254672&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000457%2Fabstract%3Frss%3Dyes Abstract: In patients with disseminated intravascular coagulation (DIC) a variety of altered coagulation parameters may be detectable, such as thrombocytopenia, prolonged global coagulation times, reduced levels of coagulation inhibitors, or high levels of fibrin split products. In addition, more sophisticated tests for activation of individual factors or pathways of coagulation may point to specific involvement of these components in the pathogenesis of the disorder. There is not a single test, however, that is sufficiently accurate to establish or reject a diagnosis of DIC. Nevertheless, a combination of widely available tests may be helpful in making the diagnosis of DIC and can also be helpful to guide in the selection of DIC patients that require specific, often expensive, interventio... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4254672 Fri, 15 Oct 2010 00:00:00 +0100 4254672 http://www.medworm.com/index.php?rid=4160575&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000299%2Fabstract%3Frss%3Dyes Abstract: Hereditary haemorrhagic telangiectasia, inherited as an autosomal dominant trait, affects approximately 1 in 5000 people. The abnormal vascular structures in HHT result from mutations in genes (most commonly endoglin or ACVRL1) whose protein products influence TGF-ß superfamily signalling in vascular endothelial cells. The cellular mechanisms underlying the generation of HHT telangiectasia and arteriovenous malformations are being unravelled, with recent data focussing on a defective response to angiogenic stimuli in particular settings. For affected individuals, there is often substantial morbidity due to sustained and repeated haemorrhages from telangiectasia in the nose and gut. Particular haematological clinical challenges include the management of severe iron deficiency ana... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4160575 Tue, 28 Sep 2010 00:00:00 +0100 4160575 http://www.medworm.com/index.php?rid=4160579&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000433%2Fabstract%3Frss%3Dyes Abstract: Increased survival in patients with β thalassaemia major (TM) allowed for several morbidities to manifest. Renal manifestations of the disease and its treatment have been poorly evaluated. There is evidence, mainly from studies in the paediatric population, of tubular dysfunction and glomerular filtration rate abnormalities in this patient population. Long-term outcomes of these changes, however, have not been prospectively investigated. The pathogenesis of these abnormalities could be attributed to iron overload, too aggressive iron removal, and/or the underlying anaemia. These changes seem to be nonprogressive, resolve spontaneously in most part, or may require iron chelator dose modifications. Relative iron depletion may explain renal function changes attributed to chelation ... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4160579 Mon, 20 Sep 2010 00:00:00 +0100 4160579 http://www.medworm.com/index.php?rid=4160578&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000421%2Fabstract%3Frss%3Dyes Abstract: The treatment of patients with relapsed and refractory Hodgkin lymphoma (HL), especially those who relapse after autologous stem cell transplantation, remains challenging. Patients with HL whose disease relapses after stem cell transplantation are rarely cured with current treatment modalities, and have a median survival of less than 3years. Since no new drugs have been approved by the FDA for HL in more than three decades, there is a clear unmet medical need for drug development for this patient population. New treatment strategies that are based on targeting oncogenic signaling pathways are currently explored. This review will focus on emerging new treatment modalities that are currently under investigation for patients with relapsed classical HL. (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4160578 Fri, 10 Sep 2010 00:00:00 +0100 4160578 http://www.medworm.com/index.php?rid=4160577&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X1000041X%2Fabstract%3Frss%3Dyes Abstract: Myeloproliferative disorders are commonly associated with thrombohemorrhagic manifestations. The current review highlights recent advances in understanding the epidemiology and pathogenetic mechanisms of thrombotic and bleeding complications. Therapeutic modalities and prophylactic interventions corresponding to the specific disease states are also discussed. (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4160577 Mon, 06 Sep 2010 00:00:00 +0100 4160577 http://www.medworm.com/index.php?rid=4160576&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000408%2Fabstract%3Frss%3Dyes Abstract: Most infants with birth weight (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=4160576 Fri, 03 Sep 2010 00:00:00 +0100 4160576 http://www.medworm.com/index.php?rid=3846609&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000287%2Fabstract%3Frss%3Dyes The authors would like to correct the omission of a reference in their discussion of the genetic interaction between Sdo1 and Tif6 in yeast. This work was published by Menne TF, Goyenechea B, Sanchez-Puig N, Wong, C. C., Tonkin, L. M., Ancliff, P. J., Brost, R. L., Costanzo, M., Boone, C., Warren, A. J.. The Shwachman–Bodian–Diamond syndrome protein mediates translational activation of ribosomes in yeast. Nat Genet. 2007;39:486–495. (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3846609 Sun, 25 Jul 2010 23:00:00 +0100 3846609 http://www.medworm.com/index.php?rid=3846603&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000275%2Fabstract%3Frss%3Dyes Abstract: Drug-induced immune hemolytic anemia (DIIHA) is rare; it can be mild or associated with acute severe hemolytic anemia (HA) and death. About 125 drugs have been implicated as the cause. The HA can be caused by drug-independent antibodies that are indistinguishable, in vitro and in vivo, from autoantibodies causing idiopathic warm type autoimmune hemolytic anemia (AIHA). More commonly, the antibodies are drug-dependent (i.e., will only react in vitro in the presence of the drug). The most common drugs to cause DIIHA are anti-microbials (e.g., cefotetan, ceftriaxone and piperacillin), which are associated with drug-dependent antibodies. The most common drug to cause AIHA is fludarabine. Finding out which drug is causing the problem and stopping that drug is the first approach to the... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3846603 Wed, 21 Jul 2010 23:00:00 +0100 3846603 http://www.medworm.com/index.php?rid=3846604&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000263%2Fabstract%3Frss%3Dyes Abstract: Anticoagulation in children is problematic for many reasons, related to the patient population as well as the anticoagulant drugs themselves. This paper describes the multitude of reasons why providing anticoagulation therapy in children is different from anticoagulation therapy in adults, and hence why dedicated paediatric anticoagulant services are the ideal structure to provide this service. The paper then describes the three most common anticoagulants used in children, and details specifically what is and is not known about them in the paediatric population. Finally the paper addresses the issue of how best to introduce newer anticoagulant drugs into the paediatric population. There remains much research to be done in this field, in the meantime clinicians need to carefully c... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3846604 Tue, 20 Jul 2010 23:00:00 +0100 3846604 http://www.medworm.com/index.php?rid=3846605&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000251%2Fabstract%3Frss%3Dyes Abstract: Immunoglobulin E (IgE) is a key mediator of anti-parasitic and anti-tumour immunity. However it is also a critical component of atopic and autoimmune diseases, and elevated serum IgE levels are a common indicator of immune dysregulation. In this review we survey the literature on genetic associations of elevated IgE in humans and mice. We find that defects in a limited number of pathways explain the majority of gene associations with IgE. Commonly, elevated IgE is associated with defects in Th bias and B cell class switching, severe T cell tolerance defects and defects in immunity at the host–environment interface. These genetic data demonstrate the mechanisms of control over IgE production and the manner in which they can be circumvented. (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3846605 Thu, 15 Jul 2010 23:00:00 +0100 3846605 http://www.medworm.com/index.php?rid=3846606&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X1000024X%2Fabstract%3Frss%3Dyes Abstract: Whether to continue oral anticoagulant therapy indefinitely after completing 3 to 6months of oral anticoagulant therapy for “unprovoked” venous thromboembolism (VTE), is one of the most important unanswered questions in VTE management. This long-term decision should be based on balancing the long-term mortality risk from recurrent VTE, largely preventable with oral anticoagulant therapy, against the long-term mortality risk of major bleeding, the principle complication of oral anticoagulant therapy. There exist important knowledge gaps in estimating the long-term mortality risk of recurrent VTE in patients with unprovoked VTE who discontinue therapy and the long-term mortality risk from major bleeding in those who continue oral anticoagulant therapy. These knowledge gaps, rev... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3846606 Wed, 14 Jul 2010 23:00:00 +0100 3846606 http://www.medworm.com/index.php?rid=3846607&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000238%2Fabstract%3Frss%3Dyes Abstract: Thrombopoietin (TPO) is the key cytokine involved in thrombopoiesis, and is the endogenous ligand for the thrombopoietin receptor that is expressed on the surface of platelets, megakaryocytes, and megakaryocytic precursors.First-generation thrombopoietic agents were recombinant forms of human TPO, and their development was discontinued after prolonged thrombocytopenia due to neutralizing auto-antibodies cross-reacting with endogenous TPO was observed. Second-generation thrombopoiesis-stimulating molecules are now available, which have unique pharmacological properties and no sequence homology to endogenous TPO. Two of these new agents, romiplostim and eltrombopag, have already completed phase III trials in primary immune thrombocytopenia and have been granted marketing authorizat... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3846607 Sun, 23 May 2010 23:00:00 +0100 3846607 http://www.medworm.com/index.php?rid=3846608&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000226%2Fabstract%3Frss%3Dyes Abstract: Over the last 17years, clinical trials conducted worldwide have demonstrated the efficacy of arsenic trioxide (As2O3) in the treatment of relapsed acute promyelocytic leukemia (APL). Currently, the role of As2O3 in front-line therapy is under investigation. Recent trials in the US have demonstrated that the addition of As2O3 to standard treatment regimens improves survival outcomes in patients with APL and may allow a reduction in cytotoxic chemotherapy exposure. As2O3 has also shown efficacy in other malignancies, particularly multiple myeloma and myelodysplastic syndromes. Therapeutic doses of As2O3 are well tolerated, with no evidence of long-term toxicity. Adverse events include APL differentiation syndrome, electrocardiographic abnormalities, and mild elevations in liver enz... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3846608 Sun, 16 May 2010 23:00:00 +0100 3846608 http://www.medworm.com/index.php?rid=3544324&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000123%2Fabstract%3Frss%3Dyes Abstract: The inherited marrow failure syndromes are a diverse set of genetic disorders characterized by hematopoietic aplasia and cancer predisposition. The clinical phenotypes are highly variable and much broader than previously recognized. The medical management of the inherited marrow failure syndromes differs from that of acquired aplastic anemia or malignancies arising in the general population. Diagnostic workup, molecular pathogenesis, and clinical treatment are reviewed. (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3544324 Sun, 25 Apr 2010 23:00:00 +0100 3544324 http://www.medworm.com/index.php?rid=3544325&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000135%2Fabstract%3Frss%3Dyes Abstract: The common autosomally inherited mucocutaneous bleeding disorder, von Willebrand disease (VWD) results from quantitative or qualitative defects in plasma von Willebrand factor (VWF). Mutation can affect VWF quantity or its functions mediating platelet adhesion and aggregation at sites of vascular damage and carrying pro-coagulant factor VIII (FVIII). Phenotype and genotype analysis in patients with the three VWD types has aided understanding of VWF structure and function. Investigation of patients with specific disease types has identified mutations in up to 70% of type 1 and 100% of type 3 VWD cases. Missense mutations predominate in type 1 VWD and act through mechanisms including rapid clearance and intracellular retention. Many mutations are incompletely penetrant and attribut... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3544325 Tue, 20 Apr 2010 23:00:00 +0100 3544325 http://www.medworm.com/index.php?rid=3544326&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000147%2Fabstract%3Frss%3Dyes Abstract: CLL is extremely heterogeneous in its clinical course, with some patients living decades with no need for treatment whilst others have a rapidly aggressive clinical course. A major focus of research has been to try to identify those biological factors that influence this heterogeneity. The goal of therapy has been to maintain the best quality of life and treat only when patients become symptomatic from their disease. For the majority of patients this means following a “watch and wait” approach to determine the rate of progression of the disease and assess for development of symptoms. Any alteration to this approach will require identification of criteria that define patients sufficiently “high-risk” that they gain benefit by introduction of early therapy. The use of molec... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3544326 Thu, 15 Apr 2010 23:00:00 +0100 3544326 http://www.medworm.com/index.php?rid=3544323&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000111%2Fabstract%3Frss%3Dyes Abstract: Advances in the molecular understanding of myeloma have led to the development of novel agents such as immunomodulatory drugs (IMiDs) and proteasome inhibitors (bortezomib). When used alone, these agents have significant activity against myeloma and responses increase significantly when they are combined with additional agents including glucocorticosteroids and chemotherapeutic agents such as alkylators. There is a drive to use these novel agents in patients with newly diagnosed myeloma, where they lead to impressive response rates with increasing duration of responses. In addition, novel agents are now the mainstays of therapy for relapsed disease. In the following paper, we summarize the key observations from recent completed and ongoing studies that determined the effect of th... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3544323 Thu, 01 Apr 2010 23:00:00 +0100 3544323 http://www.medworm.com/index.php?rid=3324556&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000032%2Fabstract%3Frss%3Dyes Summary: The pathogenesis of myelodysplastic syndromes involves a pattern of genetic, epigenetic, and immune-mediated mechanisms but little is known about what causes the specific disease features and promotes disease progression in the individual patient. The identification of JAK2 and MPL mutations, and more recently TET2, CBL and ASXL-1 mutations in these disorders provide a basis for increased understanding of disease biology and mechanisms behind progression. Such mutations are more commonly found in patients with a significant amount of marrow ring sideroblasts, and in patients belonging to the category of mixed myelodysplastic/myeloproliferative neoplasms, entities which are in focus for this review. (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3324556 Mon, 22 Feb 2010 00:00:00 +0100 3324556 http://www.medworm.com/index.php?rid=3324555&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X10000020%2Fabstract%3Frss%3Dyes Summary: There has been tremendous insight gained in the last two decades from basic science research. New molecular targets in neoplastic cells are emerging and provide the rationale for clinical development of novel agents in non-Hodgkin lymphoma. These novel agents can be broadly categorized into two groups. The first is by immunotherapy which includes novel monoclonal antibodies and immunomodulating drugs, which takes advantage of or optimizes immune system function. The other group of drugs target small molecules that may play an important role in tumorigenesis. The mechanisms of anti-tumor activity include targeting apoptotic pathways, inhibition of proteasomes, mammalian target of rapamycin (mTOR), cyclin-dependent kinases and histone deacetylases. The purpose of this review is to f... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3324555 Fri, 12 Feb 2010 00:00:00 +0100 3324555 http://www.medworm.com/index.php?rid=3324554&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000812%2Fabstract%3Frss%3Dyes Summary: Developmental Hemostasis refers to the age-related changes in the coagulation system that are most marked during neonatal life and childhood. An understanding of these changes is crucial to the accurate diagnosis of hemostatic abnormalities in neonates and children. This paper explains the current understanding of developmental hemostasis and describes the common pitfalls observed in clinical practice through failure to implement the principles into routine diagnostic work. Finally, there is a brief discussion as to a potential physiological rationale for developmental hemostasis and the implications of this for hemostatic interventions in neonates and children. There remains a need for further study to improve our understanding of the implications of developmental hemostasis in n... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3324554 Thu, 14 Jan 2010 00:00:00 +0100 3324554 http://www.medworm.com/index.php?rid=3324553&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X0900068X%2Fabstract%3Frss%3Dyes Summary: Invasive fungal infections are significant causes of morbidity and mortality in patients with hematological malignancies. Patients with acute myeloid leukemia and those who have undergone allogeneic hematopoietic stem cell transplantation are at especially high risk. Various fungal agents are responsible for this complication, but Aspergillus spp. and Candida spp. are the most frequently isolated micro-organisms; less commonly, infections could be caused by Zygomycetes or other rare molds or yeasts.Several new systemically-administered antifungal agents have been approved for clinical use since 2001; these agents include liposomal amphotericin B, voriconazole, caspofungin, and posaconazole, and they represent a major advance in antifungal therapy and have improved the prognosis of... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3324553 Thu, 07 Jan 2010 00:00:00 +0100 3324553 http://www.medworm.com/index.php?rid=3094172&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000678%2Fabstract%3Frss%3Dyes Abstract: The introduction of imatinib, a tyrosine kinase inhibitor (TKI) that targets the BCR-ABL protein, has revolutionised the treatment of chronic myeloid leukaemia (CML), producing high rates of response that have been durable in many patients. However, because of intrinsic or acquired mechanisms of imatinib resistance, in addition to the persistence of leukaemic stem cells that are resistant to imatinib-induced apoptosis, imatinib treatment does not appear to be curative. Cytogenetic and molecular monitoring enable the identification of patients showing signs of treatment failure and can be used to guide choices regarding subsequent therapeutic options, including imatinib dose escalation, treatment with a secondary TKI or, in selected cases, allogeneic stem cell transplant (allo-SCT... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3094172 Mon, 14 Dec 2009 00:00:00 +0100 3094172 http://www.medworm.com/index.php?rid=3094173&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000666%2Fabstract%3Frss%3Dyes Summary: Thrombotic thrombocytopenic purpura is an acute life threatening disorder, characterised by thrombocytopenia, microangiopathic haemolytic anaemia and multi organ microvascular thrombi that results in variable clinical symptoms. Just over a decade ago, the missing enzyme required for von Willebrand cleavage was recognised in TTP patients, subsequently identified as ADAMTS 13. Assays have confirmed that the majority of TTP cases are idiopathic and are associated with inhibitors and or IgG antibodies to ADAMTS 13. Such cases take longer to treat and are more likely to relapse. Evidence to date suggests the majority of antibodies block the spacer domain of ADAMTS 13. There may be other antibodies binding to ADAMTS 13 domains but their overall clinical involvement remains to be determi... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3094173 Mon, 07 Dec 2009 00:00:00 +0100 3094173 http://www.medworm.com/index.php?rid=3094174&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000642%2Fabstract%3Frss%3Dyes Summary: The survival prospects of critically ill patients with haematological malignancy (HM) are reviewed, as are the variables which might influence decisions about the limitation of life sustaining therapies (LLST). Approximately 40% of patients with HM admitted to ICU survive to hospital discharge and a broad admission policy is warranted. Short term survival is predicted by the severity of the underlying physiological disturbance rather than cancer specific characteristics, although the prognostic importance of neutropenia and prior stem cell transplantation remains to be clarified. Survival to hospital discharge in cancer patients following cardio-pulmonary resuscitation (CPR) is only 6–8%. Poor performance status and progressive deterioration despite ICU support appear to predict... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3094174 Mon, 16 Nov 2009 00:00:00 +0100 3094174 http://www.medworm.com/index.php?rid=3094175&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000630%2Fabstract%3Frss%3Dyes Summary: The discovery of human embryonic stem cells (hESCs) raised promises for a universal resource for cell based therapies in regenerative medicine. Recently, fast-paced progress has been made towards the generation of pluripotent stem cells (PSCs) amenable for clinical applications, culminating in reprogramming of adult somatic cells to autologous PSCs that can be indefinitely expanded in vitro. However, besides the efficient generation of bona fide, clinically safe PSCs (e.g., without the use of oncoproteins and gene transfer based on viruses inserting randomly into the genome), a major challenge in the field remains how to efficiently differentiate PSCs to specific lineages and how to select cells that will function normally upon transplantation in adults. In this review, we analyse... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3094175 Thu, 12 Nov 2009 00:00:00 +0100 3094175 http://www.medworm.com/index.php?rid=2906444&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000654%2Fabstract%3Frss%3Dyes (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2906444 Tue, 20 Oct 2009 13:23:59 +0100 2906444 http://www.medworm.com/index.php?rid=2906443&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000629%2Fabstract%3Frss%3Dyes (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2906443 Tue, 20 Oct 2009 13:23:59 +0100 2906443 http://www.medworm.com/index.php?rid=2906442&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000538%2Fabstract%3Frss%3Dyes Summary: Clinical experience since decades and numerous retro- and, recently, also prospective studies clearly demonstrate that prophylactic treatment, albeit much more expensive, is superior to on-demand treatment regardless if outcome focus on number of joint- or life-threatening bleeds or arthropathy, evaluated by X-ray or MRI, or quality of life measured by general or hemophilia specific instruments. Optimal prophylactic treatment should be started early (primary prophylaxis) but various opinions exist on the dose and dose interval, depending on the objective of treatment in the individual patient which in turn is usually dependent on the resources in the health care system. Secondary prophylaxis, started later in childhood or in adults is beneficial but less cost-effective. This revie... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2906442 Tue, 20 Oct 2009 13:23:59 +0100 2906442 http://www.medworm.com/index.php?rid=2906437&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000575%2Fabstract%3Frss%3Dyes (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2906437 Tue, 20 Oct 2009 13:23:58 +0100 2906437 http://www.medworm.com/index.php?rid=3094176&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X0900054X%2Fabstract%3Frss%3Dyes Summary: Chronic kidney disease (CKD) is a widespread health problem in the world and anemia is a common complication. Anemia conveys significant risk for cardiovascular disease, faster progression of renal failure and decreased quality of life. Patients with CKD can have anemia for many reasons, including but not invariably their renal insufficiency. These patients require a thorough evaluation to identify and correct causes of anemia other than erythropoietin deficiency. The mainstay of treatment of anemia secondary to CKD has become erythropoiesis-stimulating agents (ESAs). The use of ESAs does carry risks and these agents need to be used judiciously. Iron deficiency often co-exists in this population and must be evaluated and treated. Correction of iron deficiency can improve anemia an... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3094176 Thu, 15 Oct 2009 00:00:00 +0100 3094176 http://www.medworm.com/index.php?rid=2906439&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000435%2Fabstract%3Frss%3Dyes Summary: Atrial fibrillation is an independent risk factor for stroke and is also associated with increased mortality above the age of 65 in both males and females. Various factors increase the stroke risk, with the most widely used risk assessment tool being the CHADS2 score which incorporates, heart failure, hypertension, age, diabetes and hitroys of previous stroke or transient ischaemic attack. Despite good evidence for the effectiveness of warfarin in preventing stroke and reducing mortality in atrial fibrillation, there remain a large proportion of patients who do not receive appropriate treatment. All patients with atrial fibrillation should be considered for warfarin therapy aiming for an INR of 2.5. Whilst newer agents may replace warfarin in the future, the evidence for anti-plat... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2906439 Tue, 25 Aug 2009 23:00:00 +0100 2906439 http://www.medworm.com/index.php?rid=2906441&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000423%2Fabstract%3Frss%3Dyes Summary: ‘Monoclonal gammopathy of undetermined significance’ (MGUS) is a pre-malignant disorder characterized by limited clonal proliferation of the bone marrow plasma cells without any evidence of end-organ damage. A better understanding of the prevalence rates, natural history, and the risk factors for progression of MGUS, provides further insight into the clinical approach to management of this condition. The clinical implications of MGUS such as the risk of fracture, miscellaneous conditions associated with a monoclonal M-protein, and a practical approach to the management of MGUS patients based on a risk-stratification model are discussed in this review. (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2906441 Thu, 20 Aug 2009 23:00:00 +0100 2906441 http://www.medworm.com/index.php?rid=2906440&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000447%2Fabstract%3Frss%3Dyes Summary: Transfusion-related acute lung injury (TRALI) is the most common cause of serious morbidity and mortality due to hemotherapy. Although the pathogenesis has been related to the infusion of donor antibodies into the recipient, antibody negative TRALI has been reported. Changes in transfusion practices, especially the use of male-only plasma, have decreased the number of antibody-mediated cases and deaths; however, TRALI still occurs. The neutrophil appears to be the effector cell in TRALI and the pathophysiology is centered on neutrophil-mediated endothelial cell cytotoxicity resulting in capillary leak and ALI. This review will detail the pathophysiology of TRALI including recent pre-clinical data, provide insight into newer areas of research, and critically assess current practice... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2906440 Thu, 20 Aug 2009 23:00:00 +0100 2906440 http://www.medworm.com/index.php?rid=3094177&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000526%2Fabstract%3Frss%3Dyes The authors regret that the above article contained a minor error on page 2: the twenty first line of the right-hand column should read, “In normal red cells the ratio between reduced and oxidized glutathione (GSSG) is usually about 100:1” as opposed to “In normal red cells the ratio between oxidized and reduced glutathione (GSSH) is 100:1”. (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=3094177 Thu, 20 Aug 2009 00:00:00 +0100 3094177 http://www.medworm.com/index.php?rid=2906438&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000411%2Fabstract%3Frss%3Dyes Summary: The transfusion approach to massive hemorrhage has continually evolved since it began in the early 1900s. It started with fresh whole blood and currently consists of virtually exclusive use of component and crystalloid therapy. Recent US military experience has reinvigorated the debate on what the most optimal transfusion strategy is for patients with traumatic hemorrhagic shock. In this review we discuss recently described mechanisms that contribute to traumatic coagulopathy, which include increased anti-coagulation factors and hyperfibrinolysis. We also describe the concept of damage control resuscitation (DCR), an early and aggressive prevention and treatment of hemorrhagic shock for patients with severe life-threatening traumatic injuries. The central tenants of DCR include hy... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2906438 Wed, 19 Aug 2009 23:00:00 +0100 2906438 http://www.medworm.com/index.php?rid=2706626&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X0900040X%2Fabstract%3Frss%3Dyes Summary: In 1856 Virchow proposed a triad of causes for venous thrombosis, postulating that stasis, changes in the vessel wall or changes in the blood could lead to thrombosis. We now know that abnormally high levels of some coagulation factors and defects in the natural anticoagulants contribute to thrombotic risk. Among these, factor V Leiden, which renders factor Va resistant to activated protein C, is the most prevalent with approximately 5% of the Caucasian population having this genetic alteration. These genetically controlled variants in coagulation factors work in concert with other risk factors, such as oral contraceptive use, to dramatically increase thrombotic risk. While these abnormalities in the blood coagulation proteins are associated with thrombotic disease propensity, the... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2706626 Tue, 18 Aug 2009 10:31:05 +0100 2706626 http://www.medworm.com/index.php?rid=2706621&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000472%2Fabstract%3Frss%3Dyes (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2706621 Tue, 18 Aug 2009 10:31:04 +0100 2706621 http://www.medworm.com/index.php?rid=2706625&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000393%2Fabstract%3Frss%3Dyes Summary: Chronic lymphocytic leukaemia is a common lymphoid malignancy with a variable clinical course. While some patients never require treatment or can be managed effectively with palliative chemotherapy, others experience early disease progression and death. The development of new prognostic markers has helped in the identification of patients with high risk disease, even among those diagnosed at early stage. Recent prospective trials have established chemo-immunotherapy combinations as the new standard of care for CLL patients requiring therapy. Unfortunately, patients whose tumour cells have certain genomic aberrations, such as a chromosome 17 deletion, have a disease that is frequently refractory to conventional therapy and should have their treatment tailored accordingly. Younger p... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2706625 Wed, 29 Jul 2009 23:00:00 +0100 2706625 http://www.medworm.com/index.php?rid=2706622&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000381%2Fabstract%3Frss%3Dyes Summary: Easy bruising and bleeding are prominent features of some heritable disorders of connective tissue (HDCT), resulting from fragility of capillaries and the perivascular connective tissue rather than clotting or platelet dysfunction. The bleeding tendency is most prominent in the Ehlers–Danlos syndrome (EDS), a heterogeneous group of HDCT sharing clinical manifestations of fragility in skin, ligaments, blood vessels and internal organs. Most EDS-subtypes are caused by mutations in genes encoding fibrillar collagens type I, III or V, or genes encoding enzymes involved in the posttranslational modification of collagens. In the vascular subtype of EDS, caused by defects in type III collagen, fragility of vessel walls can lead to life-threatening bleeding and premature death. Bleeding... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2706622 Thu, 09 Jul 2009 23:00:00 +0100 2706622 http://www.medworm.com/index.php?rid=2449651&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000290%2Fabstract%3Frss%3Dyes Summary: α-Granules are essential to normal platelet activity. These unusual secretory granules derive their cargo from both regulated secretory and endocytotic pathways in megakaryocytes. Rare, inheritable defects of α-granule formation in mice and man have enabled identification of proteins that mediate cargo trafficking and α-granule formation. In platelets, α-granules fuse with the plasma membrane upon activation, releasing their cargo and increasing platelet surface area. The mechanisms that control α-granule membrane fusion have begun to be elucidated at the molecular level. SNAREs and SNARE accessory proteins that control α-granule secretion have been identified. Proteomic studies demonstrate that hundreds of bioactive proteins are released from α-granules. This breadth of pr... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2449651 Mon, 18 May 2009 04:00:00 +0100 2449651 http://www.medworm.com/index.php?rid=2449650&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000289%2Fabstract%3Frss%3Dyes This article will describe how pregnancy-associated DIC can be diagnosed promptly and how treatment should be managed strategically. It also discusses the latest developments in our understanding of haemostatic mechanisms within the placenta and how these may have relevance to new diagnostic approaches as well as novel therapeutic modalities. (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2449650 Wed, 13 May 2009 04:00:00 +0100 2449650 http://www.medworm.com/index.php?rid=2343722&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X08000672%2Fabstract%3Frss%3Dyes Summary: Therapeutic vaccines for lymphomas have been developed to induce active and long-lasting immune responses against lymphoma capable of eradicating the tumor. Most of these vaccines use the tumor B cell idiotype (the unique variable region of the surface immunoglobulin) as a tumor-specific antigen. The first human clinical trial for lymphoma vaccine was initiated 20 years ago. Along with several other phase I/II trials, it showed encouraging results which supported the initiation of three phase III trials. The results of these trials have recently been released (although not published yet) which failed to demonstrate a prolongation in progression-free survival following chemotherapy. Despite this disappointing result, a number of observations have accumulated over the years that sug... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2343722 Mon, 20 Apr 2009 15:44:40 +0100 2343722 http://www.medworm.com/index.php?rid=2343721&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X08000805%2Fabstract%3Frss%3Dyes Summary: Venous thromboembolism (VTE) is a common complication of malignancy, and is associated with significant morbidity and mortality. Anticoagulant therapy, in the form of heparin and warfarin, plays an important role in the prevention of recurrent VTE. Recent studies have demonstrated that long-term therapy with low molecular weight heparin (LMWH) is more effective than warfarin in patients with cancer. In addition, accumulating clinical evidence suggests that LMWH significantly improves overall survival in cancer patients without VTE. Intriguingly, however, this improved survival cannot simply be explained by a reduction in fatal pulmonary embolism. Furthermore, the beneficial effects persist long after the LMWH has been discontinued, suggesting that LMWH can directly influence tumou... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2343721 Mon, 20 Apr 2009 15:44:38 +0100 2343721 http://www.medworm.com/index.php?rid=2343720&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X08000817%2Fabstract%3Frss%3Dyes Summary: Globalisation may be viewed as the growing interdependence of countries worldwide through the increasing volume and variety of cross-border transactions in goods and services, and also through the more rapid and widespread diffusion of technology. Globalisation is not just an economic phenomenon, although it is frequently described as such, but includes commerce, disease and travel, and immigration, and as such it affects blood safety and supply in various ways. The relatively short travel times offered by modern aviation can result in the rapid spread of blood-borne pathogens before measures to counteract transmission can be put in place; this would have happened with SARS if the basic life cycle of the SARS virus included an asymptomatic viraemia. This risk can be amplified by e... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2343720 Mon, 20 Apr 2009 15:44:33 +0100 2343720 http://www.medworm.com/index.php?rid=2343719&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X08000799%2Fabstract%3Frss%3Dyes Summary: Bloodstream infections are an important cause of morbidity and mortality in the haematology population, and may contribute to delayed administration of chemotherapy, increased length of hospitalisation, and increased healthcare expenditure. For gram-positive, gram-negative, anaerobic and fungal infections, specific risk factors are recognised. Unique host and environmental factors contributing to pathogenesis are acknowledged in this population. Trends in spectrum and antimicrobial susceptibility of pathogens are examined, and potential contributing factors are discussed. These include the widespread use of empiric antimicrobial therapy, increasingly intensive chemotherapeutic regimens, frequent use of central venous catheters, and local infection control practices. In addition, t... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2343719 Mon, 20 Apr 2009 15:44:31 +0100 2343719 http://www.medworm.com/index.php?rid=2343718&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X08000787%2Fabstract%3Frss%3Dyes Summary: Splenomegaly is a feature of a broad range of diseases, and presents to clinicians in many fields. This review examines the aetiology of splenomegaly in the developed world, and describes a logical approach to the patient with splenomegaly. In some patients, extensive radiological and laboratory investigations will fail to yield a diagnosis: these cases of “isolated” splenomegaly are not uncommon and can be particularly challenging to manage. The risks of serious underlying disease must be balanced against the risks of invasive investigations such as splenic biopsy and diagnostic splenectomy. We discuss the options in isolated splenomegaly and their evidence base, and incorporate them into a management strategy to aid the clinician in cases of diagnostic difficulty. (Source: B... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2343718 Mon, 20 Apr 2009 15:44:29 +0100 2343718 http://www.medworm.com/index.php?rid=2343717&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X08000660%2Fabstract%3Frss%3Dyes Summary: Ferritin, a major iron storage protein, is essential to iron homeostasis and is involved in a wide range of physiologic and pathologic processes. In clinical medicine, ferritin is predominantly utilized as a serum marker of total body iron stores. In cases of iron deficiency and overload, serum ferritin serves a critical role in both diagnosis and management. Elevated serum and tissue ferritin are linked to coronary artery disease, malignancy, and poor outcomes following stem cell transplantation. Ferritin is directly implicated in less common but potentially devastating human diseases including sideroblastic anemias, neurodegenerative disorders, and hemophagocytic syndrome. Additionally, recent research describes novel functions of ferritin independent of iron storage. (Source: B... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2343717 Mon, 20 Apr 2009 15:44:25 +0100 2343717 http://www.medworm.com/index.php?rid=2706624&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000204%2Fabstract%3Frss%3Dyes Summary: Mantle cell lymphoma (MCL) is a well-defined lymphoid malignancy characterized by a rapid clinical evolution and poor response to current therapeutic protocols. The hallmark genetic alteration of MCL is the t(11;14)(q13;32) chromosomal translocation that leads to the overexpression of cyclin D1. Recently, new molecular alterations of major importance in the pathogenic mechanisms of this disease have been discovered, and have revealed the biological heterogeneity of MCL. The first section of our review discusses our current understanding of the molecular biology of this entity according to recent information from comparative genomic hybridization (CGH) and expression profiling studies, which are leading to the identification of several druggable targets. In the second section we re... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2706624 Sun, 12 Apr 2009 23:00:00 +0100 2706624 http://www.medworm.com/index.php?rid=2706623&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000174%2Fabstract%3Frss%3Dyes Summary: Large body of clinical and scientific data has been generated since the first cord blood transplantation (CBT) was performed in 1989. Superior immune plasticity of CB grafts, that allows for less stringent HLA matching, is especially valuable in the face of a persistently growing need for unrelated donor (UD) transplants. Limited cell dose remains the main setback of CBT, particularly in adult population. New strategies, such as transplantation with two cord blood units or using non-myeloablative conditioning, have remarkably expanded the availability of CB transplants in adults with hematological malignancies. Clinical trials with in vitro expanded CB-derived stem cells are under way. Currently cord blood is considered a second best choice after matched bone marrow. However, resu... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2706623 Thu, 12 Mar 2009 00:00:00 +0100 2706623 http://www.medworm.com/index.php?rid=2263898&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19282073%26dopt%3DAbstract Authors: Stanevsky A, Goldstein G, Nagler A Large body of clinical and scientific data has been generated since the first cord blood transplantation (CBT) was performed in 1989. Superior immune plasticity of CB grafts, that allows for less stringent HLA matching, is especially valuable in the face of a persistently growing need for unrelated donor (UD) transplants. Limited cell dose remains the main setback of CBT, particularly in adult population. New strategies, such as transplantation with two cord blood units or using non-myeloablative conditioning, have remarkably expanded the availability of CB transplants in adults with hematological malignancies. Clinical trials with in vitro expanded CB-derived stem cells are under way. Currently cord blood is considered a second best choice a... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2263898 Tue, 10 Mar 2009 04:00:00 +0100 2263898 http://www.medworm.com/index.php?rid=2490533&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X09000095%2Fabstract%3Frss%3Dyes Abstract: Eosinophilia is a recurrent feature and diagnostic clue in several hematologic malignancies. In stem cell- and myelopoietic neoplasms, eosinophils are derived from the malignant clone, whereas in lymphoid neoplasms and reactive states, eosinophilia is usually triggered by eosinopoietic cytokines. Myeloid neoplasms typically presenting with eosinophilia include chronic myeloid leukemia, chronic eosinophilic leukemia (CEL), other myeloproliferative neoplasms, some acute leukemias, advanced mast cell disorders, and rare forms of myelodysplastic syndromes. Diagnostic evaluations in unexplained eosinophilia have to take these diagnoses into account. In such patients, a thorough hematologic work-up including bone marrow histology and immunohistochemistry, cytogenetics, molecular marker... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2490533 Thu, 26 Feb 2009 00:00:00 +0100 2490533 http://www.medworm.com/index.php?rid=2222338&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19246139%26dopt%3DAbstract Authors: Valent P Eosinophilia is a recurrent feature and diagnostic clue in several hematologic malignancies. In stem cell- and myelopoietic neoplasms, eosinophils are derived from the malignant clone, whereas in lymphoid neoplasms and reactive states, eosinophilia is usually triggered by eosinopoietic cytokines. Myeloid neoplasms typically presenting with eosinophilia include chronic myeloid leukemia, chronic eosinophilic leukemia (CEL), other myeloproliferative neoplasms, some acute leukemias, advanced mast cell disorders, and rare forms of myelodysplastic syndromes. Diagnostic evaluations in unexplained eosinophilia have to take these diagnoses into account. In such patients, a thorough hematologic work-up including bone marrow histology and immunohistochemistry, cytogenetics, mole... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2222338 Tue, 24 Feb 2009 05:00:00 +0100 2222338 http://www.medworm.com/index.php?rid=2490532&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X08000830%2Fabstract%3Frss%3Dyes Abstract: Injury is the leading cause of death in young people and a major cause of loss of years of productive life world wide. Acute surgical care can prevent injury from turning into disability or death but requires prompt access to safe blood products to support resuscitation and restorative surgical procedures. Speed in delivering blood products is critical in resuscitation. Achieving prompt blood product support requires advanced planning and an informed balancing of risks to insure the availability of red cells and coagulation products at the time and place where they are needed. Safety and diagnostic support are critical in the post-resuscitative period where transfusion complications can delay reconstructive surgery and prolong intensive care unit stays. This paper reviews the epi... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2490532 Mon, 12 Jan 2009 00:00:00 +0100 2490532 http://www.medworm.com/index.php?rid=2104353&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19138811%26dopt%3DAbstract Authors: Murthi SB, Stansbury LG, Hess JR Injury is the leading cause of death in young people and a major cause of loss of years of productive life world wide. Acute surgical care can prevent injury from turning into disability or death but requires prompt access to safe blood products to support resuscitation and restorative surgical procedures. Speed in delivering blood products is critical in resuscitation. Achieving prompt blood product support requires advanced planning and an informed balancing of risks to insure the availability of red cells and coagulation products at the time and place where they are needed. Safety and diagnostic support are critical in the post-resuscitative period where transfusion complications can delay reconstructive surgery and prolong intensive care un... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2104353 Sat, 10 Jan 2009 05:00:00 +0100 2104353 http://www.medworm.com/index.php?rid=2490531&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.bloodreviews.com%2Farticle%2FPIIS0268960X08000829%2Fabstract%3Frss%3Dyes Summary: Pregnancy is an acquired hypercoagulable state which can lead to gestational vascular complications especially in the presence of other prothrombotic risk factors. The use of anticoagulation for prevention of pregnancy complications in women with thrombophilia is becoming more frequent. Efficacy and safety issues of therapeutic and prophylactic regimens use and monitoring of LMWH therapy in pregnancy are discussed in this review. In addition, a scoring system for women with thrombophilia is proposed which includes severity of pregnancy outcomes, thrombotic history and type of thrombophilia.Validation of this scoring system has revealed a significant correlation between the proposed score and LMWH dosages that were prescribed to the studied pregnant women.Careful diagnosis, observa... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2490531 Fri, 09 Jan 2009 00:00:00 +0100 2490531 http://www.medworm.com/index.php?rid=2100779&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19135285%26dopt%3DAbstract Authors: Sarig G, Vidergor G, Brenner B Pregnancy is an acquired hypercoagulable state which can lead to gestational vascular complications especially in the presence of other prothrombotic risk factors. The use of anticoagulation for prevention of pregnancy complications in women with thrombophilia is becoming more frequent. Efficacy and safety issues of therapeutic and prophylactic regimens use and monitoring of LMWH therapy in pregnancy are discussed in this review. In addition, a scoring system for women with thrombophilia is proposed which includes severity of pregnancy outcomes, thrombotic history and type of thrombophilia. Validation of this scoring system has revealed a significant correlation between the proposed score and LMWH dosages that were prescribed to the studied pregn... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2100779 Wed, 07 Jan 2009 05:00:00 +0100 2100779 http://www.medworm.com/index.php?rid=2046520&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19081166%26dopt%3DAbstract Authors: Farrugia A Globalisation may be viewed as the growing interdependence of countries worldwide through the increasing volume and variety of cross-border transactions in goods and services, and also through the more rapid and widespread diffusion of technology. Globalisation is not just an economic phenomenon, although it is frequently described as such, but includes commerce, disease and travel, and immigration, and as such it affects blood safety and supply in various ways. The relatively short travel times offered by modern aviation can result in the rapid spread of blood-borne pathogens before measures to counteract transmission can be put in place; this would have happened with SARS if the basic life cycle of the SARS virus included an asymptomatic viraemia. This risk can be... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2046520 Mon, 08 Dec 2008 05:00:00 +0100 2046520 http://www.medworm.com/index.php?rid=2026724&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19062140%26dopt%3DAbstract Authors: Pozo AL, Godfrey EM, Bowles KM Splenomegaly is a feature of a broad range of diseases, and presents to clinicians in many fields. This review examines the aetiology of splenomegaly in the developed world, and describes a logical approach to the patient with splenomegaly. In some patients, extensive radiological and laboratory investigations will fail to yield a diagnosis: these cases of "isolated" splenomegaly are not uncommon and can be particularly challenging to manage. The risks of serious underlying disease must be balanced against the risks of invasive investigations such as splenic biopsy and diagnostic splenectomy. We discuss the options in isolated splenomegaly and their evidence base, and incorporate them into a management strategy to aid the clinician in cases of di... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2026724 Wed, 03 Dec 2008 05:00:00 +0100 2026724 http://www.medworm.com/index.php?rid=2010917&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19046796%26dopt%3DAbstract Authors: Worth LJ, Slavin MA Bloodstream infections are an important cause of morbidity and mortality in the haematology population, and may contribute to delayed administration of chemotherapy, increased length of hospitalisation, and increased healthcare expenditure. For gram-positive, gram-negative, anaerobic and fungal infections, specific risk factors are recognised. Unique host and environmental factors contributing to pathogenesis are acknowledged in this population. Trends in spectrum and antimicrobial susceptibility of pathogens are examined, and potential contributing factors are discussed. These include the widespread use of empiric antimicrobial therapy, increasingly intensive chemotherapeutic regimens, frequent use of central venous catheters, and local infection control p... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2010917 Thu, 27 Nov 2008 05:00:00 +0100 2010917 http://www.medworm.com/index.php?rid=2010914&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D19046797%26dopt%3DAbstract Authors: Cunningham MS, Preston RJ, O'Donnell JS Venous thromboembolism (VTE) is a common complication of malignancy, and is associated with significant morbidity and mortality. Anticoagulant therapy, in the form of heparin and warfarin, plays an important role in the prevention of recurrent VTE. Recent studies have demonstrated that long-term therapy with low molecular weight heparin (LMWH) is more effective than warfarin in patients with cancer. In addition, accumulating clinical evidence suggests that LMWH significantly improves overall survival in cancer patients without VTE. Intriguingly, however, this improved survival cannot simply be explained by a reduction in fatal pulmonary embolism. Furthermore, the beneficial effects persist long after the LMWH has been discontinued, sugge... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=2010914 Thu, 27 Nov 2008 05:00:00 +0100 2010914 http://www.medworm.com/index.php?rid=1914435&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18951668%26dopt%3DAbstract Authors: Houot R, Levy R Therapeutic vaccines for lymphomas have been developed to induce active and long-lasting immune responses against lymphoma capable of eradicating the tumor. Most of these vaccines use the tumor B cell idiotype (the unique variable region of the surface immunoglobulin) as a tumor-specific antigen. The first human clinical trial for lymphoma vaccine was initiated 20 years ago. Along with several other phase I/II trials, it showed encouraging results which supported the initiation of three phase III trials. The results of these trials have recently been released (although not published yet) which failed to demonstrate a prolongation in progression-free survival following chemotherapy. Despite this disappointing result, a number of observations have accumulated ove... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1914435 Thu, 23 Oct 2008 04:00:00 +0100 1914435 http://www.medworm.com/index.php?rid=1859337&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18835072%26dopt%3DAbstract Authors: Knovich MA, Storey JA, Coffman LG, Torti SV, Torti FM Ferritin, a major iron storage protein, is essential to iron homeostasis and is involved in a wide range of physiologic and pathologic processes. In clinical medicine, ferritin is predominantly utilized as a serum marker of total body iron stores. In cases of iron deficiency and overload, serum ferritin serves a critical role in both diagnosis and management. Elevated serum and tissue ferritin are linked to coronary artery disease, malignancy, and poor outcomes following stem cell transplantation. Ferritin is directly implicated in less common but potentially devastating human diseases including sideroblastic anemias, neurodegenerative disorders, and hemophagocytic syndrome. Additionally, recent research describes novel fun... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1859337 Wed, 01 Oct 2008 04:00:00 +0100 1859337 http://www.medworm.com/index.php?rid=1825299&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18804903%26dopt%3DAbstract Authors: Shearer MJ Vitamin K deficiency bleeding (VKDB) is a rare and potentially life-threatening bleeding disorder of early infancy. Vitamin K stores are low at birth; thereafter breast-fed infants are at risk because of low concentrations in human milk. Classical VKDB occurs in the first week of life, is related to delayed or inadequate feeding and is readily prevented by small doses of vitamin K at birth. Late VKDB peaks at 3-8weeks, typically presents with intracranial haemorrhage often due to undiagnosed cholestasis with resultant malabsorption of vitamin K. Diagnosis can be difficult but PIVKA-II measurements can provide confirmation even several days post-treatment. Without vitamin K prophylaxis, the incidence of late VKDB in Europe is 4-7 cases per 10(5) births; it is higher ... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1825299 Thu, 18 Sep 2008 04:00:00 +0100 1825299 http://www.medworm.com/index.php?rid=1779062&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18774632%26dopt%3DAbstract Authors: Palumbo A, Dimopoulos M, Miguel JS, Harousseau JL, Attal M, Hussein M, Knop S, Ludwig H, von Lilienfeld-Toal M, Sonneveld P Recent studies have shown a clinical benefit of lenalidomide, an oral immunomodulatory drug, plus dexamethasone in patients with relapsed/refractory multiple myeloma (MM). The most common grade 3-4 adverse events were cytopenias, fatigue, muscle cramps, rash, infection, insomnia, and venous thromboembolism. Lenalidomide in combination with dexamethasone has been approved by the United States Food and Drug Administration and the European Medicines Agency for the treatment of patients with MM who have received at least one prior therapy. An expert panel reviewed the efficacy and toxicity of lenalidomide plus dexamethasone, and provided recommendations on th... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1779062 Fri, 05 Sep 2008 04:00:00 +0100 1779062 http://www.medworm.com/index.php?rid=1745858&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18752874%26dopt%3DAbstract Authors: McCallum L, Irvine AE CCN3, a founding member of the CCN family of growth regulators, was linked with hematology in 2003(1) when it was detected in human serum. CCN3 is expressed and secreted by hematopoietic progenitor cells in normal bone marrow. CCN3 acts through the core stem cell signalling pathways including Notch and Bone Morphogenic Protein, connecting CCN3 with the modulation of self-renewal and maturation of a number of cell lineages including hematopoietic, osteogenic and chondrogenic. CCN3 expression is disrupted in Chronic Myeloid Leukemia as a consequence of the BCR-ABL oncogene and allows the leukemic clone to evade growth regulation. In contrast, naïve cord blood progenitors undergo enhanced clonal expansion in response to CCN3. Altered CCN3 expression is ... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1745858 Mon, 25 Aug 2008 04:00:00 +0100 1745858 http://www.medworm.com/index.php?rid=1745859&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18722697%26dopt%3DAbstract Authors: Ajjan RA, Ariëns RA Atherothrombotic disease remains a major cause of mortality worldwide, and family clustering suggests an important contribution of genetic factors to disease pathogenesis. Thrombus formation represents the final step in atherothrombosis, a process influenced by genetic and environmental factors. A major difficulty of investigating the genetic regulation of thrombotic conditions is the complexity of the phenotype and the relatively modest effects of individual genetic variations. We address in this review genetic aspects involved in regulating thrombosis potential and their impact on the development of atherothrombotic disease. The effects of common genetic polymorphisms in clotting factors are discussed and examples of complex gene-gene and gene-enviro... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1745859 Thu, 21 Aug 2008 04:00:00 +0100 1745859 http://www.medworm.com/index.php?rid=1648888&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18644663%26dopt%3DAbstract Authors: Hamilton JR Arterial thrombosis, manifesting as acute myocardial infarction or ischaemic stroke, is the single most common cause of morbidity and mortality in industrialised societies. Platelets are a pre-requisite for the formation of arterial thrombi and, as a consequence, novel antiplatelet agents are sought to meet the significant clinical need for a potent, safe, and orally available therapy for the management of cardiovascular disease. Platelet thrombin receptors, termed protease-activated receptors (PARs), represent one promising candidate for the development of such therapy. This review outlines the role of platelet PARs in haemostasis and thrombosis and discusses the preclinical and clinical evidence supporting the potential of PAR antagonists as novel antiplatelet th... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1648888 Sat, 19 Jul 2008 04:00:00 +0100 1648888 http://www.medworm.com/index.php?rid=1630865&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18620786%26dopt%3DAbstract Authors: Michallet AS, Coiffier B Options for treating aggressive non-Hodgkin lymphoma (NHL) have expanded in recent years. In phase 3 clinical trials, giving rituximab with cyclophosphamide, vincristine, doxorubicin, and prednisone (CHOP) every 3 weeks (R-CHOP-21) has been associated with improved survival, without increased toxicity, in all patient groups studied. Giving dose-dense CHOP - CHOP every 2 weeks (CHOP-14) - has also proved appropriate for all patients 18-75 years old. Studies combining these approaches - dose-dense CHOP with rituximab (R-CHOP-14) - have shown improved survival over CHOP-14 in patients 60-81 years old. These results also indicate the importance of delivering chemotherapy at full dose and on schedule, which can improve survival in aggressive NHL. Effective ... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1630865 Thu, 10 Jul 2008 04:00:00 +0100 1630865 http://www.medworm.com/index.php?rid=1615815&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18617299%26dopt%3DAbstract Authors: Griesshammer M, Struve S, Barbui T The management of pregnancy in Philadelphia negative chronic myeloproliferative disorders (CMPDs) is an increasingly frequent problem. In the literature, most pregnancies are reported for women with essential thrombocythemia (ET) with about 400 pregnancies in about 200 women. In ET, first trimester abortion is the most frequent complication occurring in about one third of pregnancies. Interestingly, the incidence of maternal complications is relatively low with 3% for major thromboembolic and 2% for major bleeding events. The presence of the Jak2 mutation seems to be an independent predictor of pregnancy complications. Pregnancies in ET should be stratified according to underlying risk factors in low, high and highest risk pregnancies. Women ... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1615815 Tue, 08 Jul 2008 04:00:00 +0100 1615815 http://www.medworm.com/index.php?rid=1581840&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18599169%26dopt%3DAbstract Authors: Van Bockstaele F, Verhasselt B, Philippé J The clinical course of individual CLL patients is highly variable, with life expectancies ranging from months to decades. Importantly, a significant subset of patients presents with low grade CLL, but will nevertheless develop a more aggressive and life-threatening disease. As these patients may potentially benefit from early treatment, it is crucial to assess patients' prognosis at diagnosis, allowing individual risk-adapted therapy. Reliable predictions of prognosis in an early stage of the disease have long been lacking in the clinical workup of CLL patients. During the last decades many efforts have been made to identify prognostic markers in CLL, resulting in a plethora of reports describing the predictive value of different... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1581840 Tue, 01 Jul 2008 04:00:00 +0100 1581840 http://www.medworm.com/index.php?rid=1551206&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18583002%26dopt%3DAbstract Authors: Appelboam R, Thomas EO Spontaneous intracerebral haemorrhage is one of the most feared complications of long-term anticoagulation. Warfarin therapy not only increases the likelihood of suffering an intracranial haemorrhage, but also increases the mortality associated with it. This review aims to examine the incidence, pathogenesis, and outcome following a warfarin associated intracranial haemorrhage. It also evaluates the available evidence regarding optimal management of these patients, including timing and strategies for reversal of the coagulopathy, the role of neurocritical care and surgery, and indications for re-anticoagulation once the acute phase has past. The specific management of patients with prosthetic heart valves is also discussed. A summary of current societal ... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1551206 Tue, 24 Jun 2008 04:00:00 +0100 1551206 http://www.medworm.com/index.php?rid=1518567&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18550234%26dopt%3DAbstract Authors: Moreau P, Hulin C, Facon T Until 2007, frontline chemotherapy with melphalan and prednisone (MP) was considered as the standard of care in the treatment of elderly patients with multiple myeloma (MM). Recently, several prospective randomized studies comparing MP with the same combination plus new agents such as thalidomide (MPT) or bortezomib (MPV) clearly showed that MPT and MPV were superior to MP in terms of progression-free and overall survival. Melphalan-prednisone-lenalidomide (MPR) is currently compared to MP in one prospective trial and will also probably be superior to MP. Lenalidomide plus low-dose dexamethasone is a promising combination. Thus, at least four highly active new treatment options are now available to treat elderly patients with MM. The goal of future t... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1518567 Tue, 10 Jun 2008 04:00:00 +0100 1518567 http://www.medworm.com/index.php?rid=1508001&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18539373%26dopt%3DAbstract Authors: Martin MG, Abboud CN Acute myeloid leukemia (AML) is a disease of older adults. Efforts to intensify therapy along traditional avenues have failed to yield improved results. There remains significant clinical equipoise as to how to "induce" patients and whether or not 7+3-style regimens improve outcomes over low-dose cytarabine. What is certain is that even in those not receiving active therapy, AML is an exceptionally morbid disease. Diverse interventions are being explored in the management of older patients with AML and the currently available data will be reviewed. PMID: 18539373 [PubMed - as supplied by publisher] (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1508001 Fri, 06 Jun 2008 04:00:00 +0100 1508001 http://www.medworm.com/index.php?rid=1508002&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18538455%26dopt%3DAbstract Authors: Lee FS Idiopathic erythrocytosis is an uncommon disease, and is defined by an increase in red blood cell mass. The differential diagnosis of erythrocytosis is extensive, and can be divided into primary and secondary forms. Primary erythrocytoses are due to intrinsic defects in erythroid precursor cells and are characterized by low erythropoietin levels. Secondary erythrocytoses are extrinsic to erythroid progenitors and are characterized by either high or inappropriately normal erythropoietin levels. A distinct subset of secondary erythrocytoses are due to genetic mutations in key proteins of the oxygen-sensing pathway. These proteins constitute the core molecular machinery of oxygen-sensing with respect to red blood cell control. Apart from assigning physiologic roles for the... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1508002 Wed, 04 Jun 2008 04:00:00 +0100 1508002 http://www.medworm.com/index.php?rid=1480550&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18511163%26dopt%3DAbstract Authors: Taher AT, Otrock ZK, Uthman I, Cappellini MD Thalassemia is a congenital hemolytic disease caused by defective globin synthesis resulting in decreased quantity of globin chains. Although the life expectancy of beta-thalassemia patients has markedly improved over the last few years, patients still suffer from many complications of this congenital disease. The presence of a high incidence of thromboembolic events, mainly in beta-thalassemia intermedia, has led to the identification of a hypercoagulable state in these patients. In this paper, we review the molecular and cellular mechanisms leading to hypercoagulability in beta-thalassemia, with a special focus on thalassemia intermedia being the group with the highest incidence of thrombotic events as compared to other types of t... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1480550 Tue, 27 May 2008 04:00:00 +0100 1480550 http://www.medworm.com/index.php?rid=1439373&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18472198%26dopt%3DAbstract Authors: Shapira T, Pereg D, Lishner M The prevalence of pregnancy associated leukemia is approximately 10,000 pregnancies. This rare occurrence precludes the conducting of large, prospective studies to examine diagnostic, management and outcome issues. The treatment of a pregnant woman with leukemia may be associated with severe adverse fetal outcome including death and malformations, and therefore poses a difficult challenge for both the patient and the attending physician. Chemotherapy during the 1st trimester is associated with an increased risk for congenital malformations. However, this risk diminishes as pregnancy advances. When acute leukemia is diagnosed during the 1st trimester, patients should be treated promptly similar to non-pregnant patients. However, the aggressive indu... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1439373 Thu, 08 May 2008 04:00:00 +0100 1439373 http://www.medworm.com/index.php?rid=1422946&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18456377%26dopt%3DAbstract This article covers lymphoproliferative disorders in patients with primary or acquired immunodeficiencies. Primary immunodeficiences include Ataxia Telangiectasia and X-linked disorders such as Wiskott-Aldrich syndrome. Acquired immunodeficiencies predominantly occur in the setting of infection with the Human Immunodeficiency Virus or arise following immunosuppressive therapy administered after organ transplantation. The rising incidence of HIV throughout the world and the dramatic increase in transplant surgery since the 1990's suggest that these lymphomas will remain an important health problem. Evidence for lymphoma developing as a result of treatment with methotrexate or Tumour Necrosis Factor Antagonists for autoimmune entities will also be reviewed. The lymphoproliferations that occu... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1422946 Fri, 02 May 2008 04:00:00 +0100 1422946 http://www.medworm.com/index.php?rid=1422947&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18455284%26dopt%3DAbstract Authors: Lodewyck T, Cornelissen JJ Allogeneic hematopoietic stem cell transplantation (alloSCT) is nowadays most frequently applied in patients with acute myeloid leukemia (AML). It combines chemoradiotherapy with immunotherapy, also known as the graft-versus-leukemia (GVL) effect. While it effectively reduces the relapse rate in patients, transplanted in remission, non-relapse mortality (NRM) may counterbalance that beneficial effect. As a result, alloSCT is generally associated with a modest gain in overall survival. Therefore, alloSCT may especially be applied in patients with a relatively high risk of relapse and a relatively low risk of NRM. Here, we discuss how recent findings that have identified and validated specific prognostic factors may affect our decision making for which... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1422947 Wed, 30 Apr 2008 04:00:00 +0100 1422947 http://www.medworm.com/index.php?rid=1416142&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18448218%26dopt%3DAbstract Authors: Moreno C, Montserrat E The prognosis of patients with chronic lymphocytic leukemia (CLL) is extremely variable. Prognostication of patients with CLL has been classically based on clinical parameters. In the last few years, several biologic markers such as cytogenetics, IgVH mutations, CD38 and ZAP-70 expression in leukemic cells have shown to offer important prognostic information. However, before being incorporated into daily practice these markers require standardization and validation in prospective trials. Meanwhile, prognosis of patients with CLL should remain to be based on clinical stages and other easily obtainable clinical parameters. An important area of research is the identification of markers useful for predicting response to therapy. Among them, 17p- reflecting p... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1416142 Mon, 28 Apr 2008 04:00:00 +0100 1416142 http://www.medworm.com/index.php?rid=1399075&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18433953%26dopt%3DAbstract Authors: Craig CM, Schiller GJ Acute Myeloid Leukemia (AML) is a disorder affecting primarily elderly individuals and poses significant treatment challenges. Much has been learned about the underlying immunologic, cytogenetic and molecular features of AML in recent years, and many features have been identified that portend a poor prognosis for elderly patients with newly diagnosed AML. Despite this, treatment outcomes for elderly patients remain poor for both newly diagnosed and relapsed disease. While conventional treatment approaches may be appropriate for some elderly patients, the vast majority do not tolerate intensive chemotherapy well, thus alternative strategies have been investigated. Here we review both conventional and novel treatment approaches for elderly patients with AML... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1399075 Mon, 21 Apr 2008 04:00:00 +0100 1399075 http://www.medworm.com/index.php?rid=1399074&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18433954%26dopt%3DAbstract Authors: Roberts I, Stanworth S, Murray NA Thrombocytopenia is one of the commonest haematological problems in neonates, affecting at least 25% of all admissions to neonatal intensive care units (NICUs) [Murray NA, Howarth LJ, McCloy MP et al. Platelet transfusion in the management of severe thrombocytopenia in neonatal intensive care unit patients. Transfus Med 2002;12:35-41; Garcia MG, Duenas E, Sola MC et al. Epidemiologic and outcome studies of patients who received platelet transfusions in the neonatal intensive care unit. J Perinatol 2001;21:415-20; Del Vecchio A, Sola MC, Theriaque DW et al. Platelet transfusions in the neonatal intensive care unit: factors predicting which patients will require multiple transfusions. Transfusion 2001;41:803-8]. Although a long list of disorders... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1399074 Mon, 21 Apr 2008 04:00:00 +0100 1399074 http://www.medworm.com/index.php?rid=1396086&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18430498%26dopt%3DAbstract Authors: Brissot P, Troadec MB, Bardou-Jacquet E, Lan CL, Jouanolle AM, Deugnier Y, Loréal O Iron overload diseases of genetic origin are an ever changing world, due to major advances in genetics and molecular biology. Five major categories are now established: HFE-related or type1 hemochromatosis, frequently found in Caucasians, and four rarer diseases which are type 2 (A and B) hemochromatosis (juvenile hemochromatosis), type 3 hemochromatosis (transferrin receptor 2 hemochromatosis), type 4 (A and B) hemochromatosis (ferroportin disease), and a(hypo)ceruloplasminemia. Increased duodenal iron absorption and enhanced macrophagic iron recycling, both due to an impairment of hepcidin synthesis, account for the development of cellular excess in types 1, 2, 3, and 4B hemochromatosis ... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1396086 Fri, 18 Apr 2008 04:00:00 +0100 1396086 http://www.medworm.com/index.php?rid=1382256&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18417261%26dopt%3DAbstract Authors: Uthman I, Godeau B, Taher A, Khamashta M Various hematological pathologies have been described in association with antiphospholipid syndrome (APS). Thrombocytopenia is frequently found in APS patients, its incidence has ranged from 22-42% in different series, it is usually moderate (>50x10(9)/L) without clinical manifestation and requires no intervention. A high percentage of patients with isolated idiopathic thrombocytopenic purpura have antiphospholipid antibodies, however the pathogenetic role and the clinical importance of these antibodies in this condition is still not clear. Other hematological manifestations reported in association with APS include: bone marrow necrosis, and various thrombotic microangiopathic syndromes such as: thrombotic thrombocytopenic purpura, h... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1382256 Mon, 14 Apr 2008 04:00:00 +0100 1382256 http://www.medworm.com/index.php?rid=1310874&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18343002%26dopt%3DAbstract Authors: Firpi RJ, Nelson DR Viral hepatitis is the third major cause of liver dysfunction in allogeneic transplant recipients and has become a significant concern in patients with hematological malignancies receiving chemotherapy. Thus, identification of patients at risk for viral hepatitis is very important when evaluating and treating hematological malignancies. Serologic screening for all patients should include anti-HCV, hepatitis B surface antigen (HBsAg), hepatitis B surface antibody (anti-HBs), and hepatitis B core antibody (anti-HBc) testing. Current therapies for hepatitis B (HBV) virus infection are aimed at viral suppression, while treatment for hepatitis C (HCV) virus can eradicate infection in many treated patients. To prevent HBV viral reactivation, prophylaxis with nucl... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1310874 Thu, 13 Mar 2008 04:00:00 +0100 1310874 http://www.medworm.com/index.php?rid=1194678&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18234403%26dopt%3DAbstract Authors: D'Andrea G, D'Ambrosio R, Margaglione M Oral anticoagulants, the main drugs used for the prevention and treatment of thromboembolic diseases, exhibit a greater than 10-fold inter-individual variability in the dose requirement to achieve a therapeutic response. The relationship between the dose prescribed and the individual response is regulated by genetic and environmental factors. In particularly, molecular analysis of two genes, encoding for the enzyme responsible for the warfarin (S)-isoform catabolism (CYP2C9) and for the target enzyme vitamin K epoxide reductase complex 1 (VKORC1), strongly suggested that their genetic variations greatly affect the individual response to oral anticoagulants. Genotype based modelling explained a large amount of dose-variations. As a perspe... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1194678 Mon, 28 Jan 2008 05:00:00 +0100 1194678 http://www.medworm.com/index.php?rid=1188470&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18226435%26dopt%3DAbstract Authors: Watson HG, Chee YL While there is good evidence for a protective effect of aspirin against occlusive vascular events in individuals with arterial disease, its role in preventing venous thromboembolism (VTE) is unclear. In this article we review the role of aspirin and other antiplatelet drugs in prevention of venous thromboembolism in surgical patients, high risk medical patients requiring aspirin for other reasons, patients with myeloproliferative disorders, long distance travellers and patients receiving treatment with the IMiD class of drugs. Overall, data from the PEP study and Anti-Platelet Trialists' systematic review show that aspirin reduces the risk of VTE by around 25% in high risk surgical patients. Data from retrospective and before/after studies also suggest effic... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1188470 Mon, 14 Jan 2008 05:00:00 +0100 1188470 http://www.medworm.com/index.php?rid=1139647&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18180086%26dopt%3DAbstract Authors: Salles II, Feys HB, Iserbyt BF, De Meyer SF, Vanhoorelbeke K, Deckmyn H Inherited platelet disorders constitute a large group of diseases involving a wide range of genetic defects that can lead to bleeding symptoms of varying severity. They are associated with defects in surface membrane glycoproteins resulting in e.g. Bernard Soulier Syndrome and Glanzmann Thrombasthenia causing defects in platelet adhesion and aggregation, respectively, as well as in receptors for agonists (a.o. P2Y(12), TXA(2)) disrupting platelet signalling. Defects affecting platelet granules can be characterised by abnormalities of alpha-granules as in the Gray platelet syndrome or dense granules as in Hermansky-Pudlak and Chediak-Higashi syndromes, the latter two also altering other cytoplasmic organell... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1139647 Wed, 02 Jan 2008 05:00:00 +0100 1139647 http://www.medworm.com/index.php?rid=1124077&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18164793%26dopt%3DAbstract Authors: Dokal I, Vulliamy T The inherited aplastic anaemias/bone marrow (BM) failure syndromes are a heterogeneous group of disorders characterized by BM failure usually in association with one or more somatic abnormality. The BM failure often presents in childhood but this may not be until adulthood in some cases highlighting the need for the adult haematologist to be aware of these disorders. Indeed some patients initially labelled as "idiopathic aplastic anaemia" are cryptic presentations of these genetic syndromes. Since 1992, when the first Fanconi anaemia (FA) gene was cloned there have been considerable advances in the genetics of these syndromes. These advances are beginning to provide a better understanding of normal haemopoiesis and how this might be disrupted in patients wi... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1124077 Fri, 28 Dec 2007 05:00:00 +0100 1124077 http://www.medworm.com/index.php?rid=1085467&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18068281%26dopt%3DAbstract Authors: Hellström-Lindberg E, Malcovati L Treatment of myelodysplastic syndromes (MDS) has evolved to encompass a broad spectrum of therapies aiming to inhibit apoptosis, promote hemopoiesis, and reduce proliferation of clonal immature cells. A small but expanding cohort of patients with MDS may be cured, but for the majority the aim of treatment is to prolong survival and to improve quality of life. Patients with low-risk MDS mainly suffer from the effects of severe anemia and an important therapeutic goal is to maintain acceptable hemoglobin levels by optimal transfusion regimens or by erythropoietin+/-granulocyte-colony-stimulating factor, which normalizes hemoglobin levels or abolish transfusion need in around 40% of patients. Lenalidomide has emerged as a drug of choice for ... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1085467 Thu, 06 Dec 2007 05:00:00 +0100 1085467 http://www.medworm.com/index.php?rid=1080289&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18063459%26dopt%3DAbstract Authors: Brodsky RA PNH is an uncommon acquired hemolytic anemia that often manifests with hemoglobinuria, abdominal pain, smooth muscle dystonias, fatigue, and thrombosis. The disease results from the expansion of hematopoietic stem cells harboring a mutation in a gene, PIG-A, that is required for the biosynthesis of a lipid moiety, glycosylphosphatidylinositol (GPI), that attaches dozens of different proteins to the cell surface. Thus, PNH cells are deficient in cell surface GPI anchored proteins; this deficiency on erythrocytes leads to intravascular hemolysis since certain GPI anchored proteins normally function as complement regulators. Free hemoglobin released from intravascular hemolysis leads to circulating nitric oxide depletion and is responsible for many of the clinical mani... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1080289 Fri, 30 Nov 2007 05:00:00 +0100 1080289 http://www.medworm.com/index.php?rid=1057221&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D18039551%26dopt%3DAbstract Authors: Lucarelli G, Gaziev J Hematopoietic stem cell transplantation (HSCT) remains the only curative option for patients with thalassemia. Current results of transplantation in patients aged less than 17 years from matched related donors offer 80% to 87% probability of cure according to risk classes. Adult thalassemics treated with myeloablative conditioning continue to have inferior results because of their advanced stage of disease. With the introduction of high-resolution tissue typing techniques transplant centres worldwide are able to offer allogeneic HSCT to a much larger cohort of patients who could not benefit from transplantation because of lack of matched family donor. Although limited number of patients treated, results of transplant from unrelated matched donors are comp... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1057221 Sat, 24 Nov 2007 05:00:00 +0100 1057221 http://www.medworm.com/index.php?rid=1007124&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D17981381%26dopt%3DAbstract Authors: Bussel JB, Primiani A Fetal and neonatal alloimmune thrombocytopenia (AIT) is a result of a parental incompatibility of platelet-specific antigens and the transplacental passage of maternal alloantibodies against the platelet antigen shared by the father and the fetus. It occurs in approximately 1 in 1000 live births and is the most common cause of severe thrombocytopenia in fetuses and term neonates. As screening programs are not routinely performed, most affected fetuses are identified after birth when neonatal thrombocytopenia is recognized. In severe cases, the affected fetus is identified as a result of suffering from an in utero intracranial hemorrhage. Once diagnosed, AIT must be treated antenatally as the disease can be more severe in subsequent pregnancies. While ther... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=1007124 Fri, 02 Nov 2007 04:00:00 +0100 1007124 http://www.medworm.com/index.php?rid=989586&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D17964017%26dopt%3DAbstract Authors: Urbanus RT, Derksen RH, de Groot PG The diagnosis of the antiphospholipid syndrome, a non-inflammatory autoimmune disease characterized by thrombosis or pregnancy morbidity in the presence of antiphospholipid antibodies, depends greatly upon laboratory diagnostics. The diagnostic value of all available assays to detect antiphospholipid antibodies and the anticardiolipin assay in particular, is a matter of ongoing debate. Although the presence of lupus anticoagulant correlates best with thrombosis, accurate determination is not always possible due to anticoagulant treatment. Data on the predictive value of alternatives such as the anti-beta(2)-glycoprotein I and the anti-prothrombin antibody assay are insufficient and prospective cohort studies are needed. Determining antiphosp... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=989586 Wed, 24 Oct 2007 04:00:00 +0100 989586 http://www.medworm.com/index.php?rid=921552&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D17904258%26dopt%3DAbstract Authors: Petz LD The cold antibody autoimmune hemolytic anemias (AIHAs) are primarily comprised of cold agglutinin syndrome (CAS) and paroxysmal cold hemoglobinuria (PCH) but, in addition, there are unusual instances in which patients satisfy the serologic criteria of both warm antibody AIHA and CAS ("mixed AIHA"). CAS characteristically occurs in middle-aged or elderly persons, often with signs and symptoms exacerbated by cold. The responsible antibody is of the IgM immunoglobulin class, is maximally reactive in the cold but with reactivity up to at least 30 degrees C. Therapy is often ineffective, but newer agents such as rituximab have been beneficial in some patients. PCH occurs primarily in children, often after an upper respiratory infection. The causative antibody is of the IgG ... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=921552 Wed, 26 Sep 2007 04:00:00 +0100 921552 http://www.medworm.com/index.php?rid=921551&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D17904259%26dopt%3DAbstract Authors: Packman CH The diagnosis of autoimmune hemolytic anemia (AHA) requires evidence of shortened red blood cell (RBC) survival mediated by autoantibodies directed against autologous RBCs. About 80 percent of patients with AHA have warm-reactive antibodies of the IgG isotype; the remainder exhibit cold-reactive autoantibodies. Typical patients exhibit anemia, reticulocytosis, spherocytes and polychromasia on the blood film and a positive direct antiglobulin test (DAT). Increased indirect serum bilirubin, urinary urobilinogen and serum lactate dehydrogenase (LDH), and decreased serum haptoglobin are not required for the diagnosis, but are frequently present. Patients with AHA and no underlying associated disease are said to have primary or idiopathic AHA. AHA in patients with associ... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=921551 Wed, 26 Sep 2007 04:00:00 +0100 921551 http://www.medworm.com/index.php?rid=903130&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D17889973%26dopt%3DAbstract Authors: Unwin RD, Whetton AD Proteomics technologies are emerging as a useful tool in the identification of disease biomarkers, and in defining and characterising both normal physiological and disease processes. Many cellular changes in protein expression in response to an external stimulus or mutation can only be characterised at the proteome level. In these cases protein expression is often controlled by altered rates of translation and/or degradation, making proteomics an important tool in the analysis of biological systems. In the leukaemias, post-translational modification of proteins (e.g. phosphorylation, acetylation) plays a key role in the molecular pathology of the disease: such modifications can now be detected with novel proteomic methods. In a clinical setting, serum rema... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=903130 Fri, 21 Sep 2007 04:00:00 +0100 903130 http://www.medworm.com/index.php?rid=870282&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D17850941%26dopt%3DAbstract Authors: Dispenzieri A POEMS syndrome is a rare paraneoplastic syndrome secondary to a plasma cell dyscrasia. Recognition of the complex of a combination of peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasmaproliferative disorder, skin changes, papilledema, extravascular volume overload (peripheral edema, pleural effusions, ascites), sclerotic bone lesions, thrombocytosis, Castleman disease is the first step in effectively managing the disease. A rise in the blood levels of vascular endothelial growth factor is usually confirmatory. More than 95% of patients will have monoclonal lambda sclerotic plasmacytoma(s) or bone marrow infiltration. In patients with a dominant sclerotic plasmacytoma, first line therapy should include radiation to the lesion. Retrospective ana... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=870282 Mon, 10 Sep 2007 04:00:00 +0100 870282 http://www.medworm.com/index.php?rid=849761&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D17804128%26dopt%3DAbstract Authors: Vamvakas EC, Blajchman MA Allogeneic blood transfusion (ABT)-related immunomodulation (TRIM) encompasses the laboratory immune aberrations that occur after ABT and their established or purported clinical effects. TRIM is a real biologic phenomenon resulting in at least one established beneficial clinical effect in humans, but the existence of deleterious clinical TRIM effects has not yet been confirmed. Initially, TRIM encompassed effects attributable to ABT by immunomodulatory mechanisms (e.g., cancer recurrence, postoperative infection, or virus activation). More recently, TRIM has also included effects attributable to ABT by pro-inflammatory mechanisms (e.g., multiple-organ failure or mortality). TRIM effects may be mediated by: (1) allogeneic mononuclear cells; (2) white-b... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=849761 Mon, 03 Sep 2007 04:00:00 +0100 849761 http://www.medworm.com/index.php?rid=835908&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D17761373%26dopt%3DAbstract This report highlights some of the key recent findings in multiple myeloma, and describes areas for future research. PMID: 17761373 [PubMed - as supplied by publisher] (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=835908 Tue, 28 Aug 2007 04:00:00 +0100 835908 http://www.medworm.com/index.php?rid=790483&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D17683834%26dopt%3DAbstract Authors: Cashen AF, Bartlett NL The majority of patients who are diagnosed with Hodgkin lymphoma (HL) will be cured with primary chemotherapy. For those who relapse, autologous stem cell transplantation (ASCT) has become the standard of care. Randomized clinical trials have demonstrated that approximately 50% of patients with chemosensitive relapsed HL can achieve long term disease free survival with ASCT. However, optimal therapy of those who have chemorefractory disease or who relapse after an ASCT has not been established. Reduced intensity allogeneic stem cell transplantation may benefit these patients, although a definite graft versus HL effect has not been demonstrated and treatment-related mortality remains relatively high. New salvage regimens that incorporate gemcitabine, vino... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=790483 Tue, 31 Jul 2007 04:00:00 +0100 790483 http://www.medworm.com/index.php?rid=717906&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D17611006%26dopt%3DAbstract Authors: Mason PJ, Bautista JM, Gilsanz F Deficiency of glucose-6-phosphate dehydrogenase is a very common X-linked genetic disorder though most deficient people are asymptomatic. A number of different G6PD variants have reached polymorphic frequencies in different parts of the world due to the relative protection they confer against malaria infection. People, usually males, with deficient alleles are susceptible to neonatal jaundice, and acute hemolytic anemia, usually during infection, after treatment with certain drugs or after eating fava beans. Very rarely de novo mutations can arise causing the more severe condition of chronic nonspherocytic hemolytic anemia. Altogether 160 different mutations have been described. The majority of mutations cause red cell enzyme deficiency by decr... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=717906 Mon, 02 Jul 2007 04:00:00 +0100 717906 http://www.medworm.com/index.php?rid=696712&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D17590250%26dopt%3DAbstract Authors: Janka GE Hemophagocytic syndromes (hemophagocytic lymphohistiocytosis, HLH) represent a severe hyperinflammatory condition with the cardinal symptoms prolonged fever, cytopenias, hepatosplenomegaly, and hemophagocytosis by activated, morphologically benign macrophages. Biochemical markers include elevated ferritin and triglycerides, and low fibrinogen. Whereas in children several inherited immune deficiencies may lead to this syndrome, most adults with HLH have no known underlying immune defect. Nevertheless, impaired function of natural killer (NK) cells and cytotoxic T-cells (CTL) is characteristic for both genetic and acquired forms of HLH. Frequent triggers are infectious agents, mostly viruses of the herpes group. Malignant lymphomas, especially in adults, may be associat... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=696712 Wed, 20 Jun 2007 04:00:00 +0100 696712 http://www.medworm.com/index.php?rid=628697&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D17512649%26dopt%3DAbstract Authors: Savage KS Peripheral T-cell lymphomas (PTCLs) are a biologically diverse and uncommon group of diseases. Compared to their B-cell counterparts, PTCLs remain largely unexplored and the optimal treatment ill-defined due to disease rarity and biological heterogeneity. With the notable exception of ALK-pos anaplastic large cell lymphoma (ALCL), the prognosis of most PTCL subtypes is extremely is poor with a 5 y overall survival of approximately 15-30% in most series. The international prognostic index has been useful in defining different risk groups within some PTCL subtypes, including PTCL unspecified (PTCLU). Attempts have been made to define disease subgroups within the biologically heterogeneous PTCLU based on T-helper chemokine receptor profile and/or gene expression profili... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=628697 Thu, 17 May 2007 04:00:00 +0100 628697 http://www.medworm.com/index.php?rid=487094&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D17367905%26dopt%3DAbstract Authors: Rajkumar SV, Lacy MQ, Kyle RA Monoclonal gammopathy of undetermined significance (MGUS) and smoldering multiple myeloma (SMM) are asymptomatic disorders characterized by monoclonal plasma cell proliferation in the bone marrow in the absence of end-organ damage. Updated diagnostic criteria for these disorders, risk-stratification models to determine prognosis, and the current management of these two entities are discussed in this review. PMID: 17367905 [PubMed - as supplied by publisher] (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=487094 Thu, 15 Mar 2007 04:00:00 +0100 487094 http://www.medworm.com/index.php?rid=475880&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D17360088%26dopt%3DAbstract Authors: Zanella A, Fermo E, Bianchi P, Chiarelli LR, Valentini G Red cell pyruvate kinase (PK) deficiency is the most frequent enzyme abnormality of glycolysis causing chronic non-spherocytic haemolytic anaemia. The disease is transmitted as an autosomal recessive trait, clinical symptoms usually occurring in compound heterozygotes for two mutant alleles and in homozygotes. The severity of haemolysis is highly variable, ranging from very mild or fully compensated forms to life-threatening neonatal anaemia necessitating exchange transfusions. Erythrocyte PK is synthesised under the control of the PK-LR gene located on chromosome 1. One hundred eighty different mutations in PK-LR gene, mostly missense, have been so far reported associated to PK deficiency. First attempts to delineate th... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=475880 Mon, 12 Mar 2007 04:00:00 +0100 475880 http://www.medworm.com/index.php?rid=436761&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D17293017%26dopt%3DAbstract This article reviews the clinical experience with tipifarnib and lonafarnib in the treatment of hematologic malignancies. PMID: 17293017 [PubMed - as supplied by publisher] (Source: Blood Reviews) Blood Reviews journals http://www.medworm.com/rss/comments.php?id=436761 Fri, 09 Feb 2007 07:00:00 +0100 436761 http://www.medworm.com/index.php?rid=436762&cid=s_34569_19_f&fid=34569&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fentrez%2Fquery.fcgi%3Ftmpl%3DNoSidebarfile%26db%3DPubMed%26cmd%3DRetrieve%26list_uids%3D17289231%26dopt%3DAbstract Authors: Tedeschi A, Baratè C, Minola E, Morra E Cryoglobulinemia refers to the presence in serum of immunoglobulins that precipitate at a cold temperature. Type I cryoglobulins are single monoclonal immunoglobulins usually associated with haematological disorders. Types II and III are mixed cryoglobulins, composed of monoclonal or polyclonal IgM respectively, having rheumatoid factor activity that bind to polyclonal immunoglobulins. Mixed cryoglobulinemia (MC) syndrome is a consequence of immune-complex mediated vasculitis and is characterized by a typical clinical triad: purpura, weakness, arthralgias; many organs particularly kidney and peripheral nervous system may be involved. MC may be associated with infectious and systemic disorders and since 1990 studies have demonstrated tha... Blood Reviews journals http://www.medworm.com/rss/comments.php?id=436762 Tue, 06 Feb 2007 07:00:00 +0100 436762